Circulatory System

10
Diseases and Conditions of the

Circulatory System
CHAPTER OUTLINE
Orderly Function of the Circulatory System, 495 Cardiac Tamponade, 539
Cardiovascular Diseases, 498 Vascular Conditions, 541
Lymphatic and Blood Disorders, 499 Emboli, 543
Arteriosclerosis, 546
Coronary Artery Disease, 499
Aneurysms, 547
Angina Pectoris, 503
Phlebitis, 549
Myocardial Infarction, 504
Thrombophlebitis, 549
Cardiac Arrest, 508 Varicose Veins, 550
Hypertensive Heart Disease, 509 Thromboangiitis Obliterans (Buerger
Essential Hypertension, 510 Disease), 551
Malignant Hypertension, 512 Raynaud Disease, 552
Lymphatic and Blood Disorders, 513 Blood Dyscrasias, 553
Congestive Heart Failure, 513 Anemias, 554
Agranulocytosis, 559
Cor Pulmonale, 515 Polycythemia, 560
Pulmonary Edema, 517 Thrombocytopenia, 560
Cardiomyopathy, 519 Leukemias, 560
Pericarditis, 520 Acute Lymphocytic Leukemia, 561
Chronic Lymphocytic Leukemia, 562
Myocarditis, 522
Acute Myelogenous Leukemia, 563
Endocarditis, 523 Chronic Myelogenous Leukemia, 564
Rheumatic Fever, 525 Lymphatic Diseases, 565
Rheumatic Heart Disease, 526 Lymphedema, 566
Valvular Heart Disease, 528 Lymphangitis, 568
Mitral Stenosis, 528 Lymphoma, 569
Mitral Insufficiency, 529 Transfusion Incompatibility Reaction, 572
Mitral Valve Prolapse, 530
Clotting Disorders, 574
Arrhythmias, 530 Classic Hemophilia, 574
Shock, 534 Disseminated Intravascular Coagulation, 575
Cardiogenic Shock, 539
493
494 C HA P T E R 10 Diseases and Conditions of the Circulatory System
LEARNING OBJECTIVES
After studying Chapter 10, you should be able to: 16. Name the causes of cardiac arrhythmias.
1. Discuss the anatomy and physiology of the heart. 17. Discuss treatment options for cardiac
2. Name the common presenting symptoms in arrhythmias.
patients with cardiovascular disease. 18. Describe the signs and symptoms of shock.
3. Describe the pathology of coronary artery disease 19. Discuss cardiac tamponade.
(CAD). 20. Explain the possible consequences of emboli.
4. Name the contributing factors for coronary artery 21. Compare arteriosclerosis with atherosclerosis.
disease. 22. Describe an aneurysm, and explain how it is
5. Explain what causes the pain of angina pectoris. diagnosed.
6. Explain the difference between angina pectoris 23. Explain the treatment for (a) thrombophlebitis
and myocardial infaction (MI). and (b) varicose veins.
7. Describe the treatment of MI and cardiac arrest. 24. Describe the vascular pathology of Raynaud
8. Name and describe the symptoms of the most disease.
prevalent cardiovascular disorder in the United 25. Define anemia, and list the presenting
States. symptoms.
9. Explain what happens when the pumping action 26. Describe how anemias are classified and give
of the heart fails. some examples.
10. Compare left-sided heart failure with right-sided 27. State the causes of agranulocytosis.
heart failure. 28. Describe the typical symptoms in all types of
11. Name some causes of cardiomyopathy. leukemias.
12. Distinguish among pericarditis, myocarditis, and 29. Distinguish between lymphedema and
endocarditis. lymphangitis.
13. Explain why rheumatic fever is considered a 30. Explain the diagnostic significance of Reed-
systemic disease. Sternberg cells in lymphoma.
14. Recall the cardiac manifestations of rheumatic 31. Name the signs and symptoms of transfusion
heart disease. incompatibility reaction.
15. Explain the pathophysiology of valvular heart 32. Explain the cause of classic hemophilia.
disease. 33. Describe disseminated intravascular coagulation
KEY TERMS
agglutination (ah-glue-tih-NAY-shun) hemolytic (hem-oh-LIT-ik)
aggregation (ag-reh-GAY-shun) hypovolemia (high-poh-voh-LEE-mee-ah)
angioplasty (AN-jee-oh-plas-tee) hypoxia (high-POX-see-ah)
arteriosclerosis (ar-tee-ree-oh-skleh-ROW-sis) ischemia (is-KEY-mee-ah)
asystole (a-SIS-toh-lee) orthopnea (or-THOP-nee-ah)
atherosclerosis (ath-er-oh-skleh-ROW-sis) perfusion (per-FYOU-zhun)
bradycardia (brady-KAR-dee-ah) petechiae (pee-TEE-kee-ee)
bruit (BREW-ee) phlebotomy (phleh-BOT-oh-mee)
cardiomegaly (car-dee-oh-MEG-ah-lee) plaque (PLACK)
cardiomyopathy (car-dee-oh-my-OP-ah-thee) purpura (PUR-pu-rah)
cellulitis (sell-u-LIE-tis) syncope (SIN-ko-pee)
dyscrasia (dis-CRAY-zee-ah) tachycardia (tack-ee-CAR-dee-ah)
ecchymosis (ech-ih-MO-sis) tamponade (tam-pon-ADE)
embolism (EM-boh-lizm) thrombus (THROM-bus)
hematopoiesis (hem-ah-toh-poy-EE-sis)
CHAPTER 10 Diseases and Conditions of the Circulatory System 495
through the pulmonary valve and, with ventricular

ORDERLY FUNCTION OF THE contraction, into the pulmonary arteries and on to the
CIRCULATORY SYSTEM lungs. In the lungs, carbon dioxide is removed and
oxygen is added to the blood. Freshly oxygenated
Circulation of blood to the organs and tissues of the blood then returns to the heart via the pulmonary
body is the primary function of the circulatory system. veins. It enters the left atrium, moves through the
The heart is at the center of the circulatory system mitral (bicuspid) valve with atrial contraction, and
(Figures 10-1 and 10-2). Its steady beating pumps enters the left ventricle. As the left ventricle contracts,
about 5 quarts of blood through a complete vascular the blood is forced through the aortic valve, into the
circuit of the body every minute in an adult; this is aorta, and on to the rest of the body (Figures 10-3 and
called the cardiac cycle. This circuit comprises a 10-4). This process is called the cardiac cycle (Figure
network of vessels: the arteries, veins, and capillaries 10-5). Refer to Chapter 2 for a description of fetal
(Figure 10-3). circulation.
The heart consists of two side-by-side pumps, each The heart is enclosed by the double-layered peri-
divided into two chambers: two upper chambers cardium, which is composed of an inner serous layer
called atria, and two lower chambers called ventricles. (visceral pericardium or epicardium) and an outer
As venous blood returns to the heart from the body, fibrous layer (parietal pericardium). Between these
it enters the right atrium, passes through the tricuspid layers in the pericardial cavity is a small amount of
valve, and with atrial contraction, enters the right serous fluid that reduces friction during cardiac move-
ventricle. Heart valves prevent the blood from flowing ments. Cardiac muscle tissue or myocardium is com-
backward. From the right ventricle, blood is pumped posed of striated muscle cells that can contract
Left common carotid artery
Left subclavian artery

Brachiocephalic trunk
Arch of aorta
Superior vena cava
Ligamentum arteriosum
Ascending aorta
Auricle of left atrium
Pulmonary trunk
Left pulmonary veins
Right pulmonary veins Great cardiac vein
Auricle of right atrium Circumflex artery
Anterior interventricular branches of

left coronary artery and cardiac vein
Right coronary artery
(in interventricular sulcus)
and cardiac vein
Left ventricle
Right ventricle S
R L
Apex
I
• Figure 10-1 The heart and great vessels, anterior view. (From Patton KT, Thibodeau GA: Anatomy and
physiology, ed 9, St Louis, 2016, Mosby.)
Left common carotid artery
Left subclavian artery Brachiocephalic trunk
Aortic arch
Superior vena cava

Left pulmonary artery
Right pulmonary artery
Left pulmonary veins Right pulmonary veins
Auricle of left atrium

Left atrium Right atrium
Inferior vena cava

Great cardiac vein
(in coronary sulcus) Coronary sinus
Posterior artery and Posterior interventricular branch of

vein of left ventricle right coronary artery
(in interventricular sulcus)
Left ventricle
Middle cardiac vein
Posterior
interventricular sulcus Right ventricle S
L R
Apex
I
• Figure 10-2 The heart and great vessels, posterior view. (From Patton KT, Thibodeau GA: Anatomy
and physiology, ed 9, St Louis, 2016, Mosby.)
Left
atrium
Right
ventricle Left ventricle
• Figure 10-3 Circulation through the body.

Superior
Right vena cava Left
Aorta pulmonary
pulmonary
arteries arteries
Left
pulmonary
Right veins
pulmonary
veins
Mitral
Pulmonary valve
valve
Tricuspid Aortic
valve valve
Right Left
ventricle ventricle
Inferior Interventricular
vena cava septum
• Figure 10-4 Circulation through the heart.
1. DIASTOLE 2. DIASTOLE 3. SYSTOLE BEGINS

• Atria fill • Increased atrial pressure • Atria contract and empty
• All valves closed opens AV valves • Ventricles are full
• Ventricles fill
6. DIASTOLE 5. SYSTOLE 4. SYSTOLE

• Ventricles empty • Ventricles contract • Ventricles begin contraction
• Ventricles relax • Increased pressure in ventricles • Pressure closes
• Aortic and pulmonary • Aortic and pulmonary valves open AV valves
valves close • Blood ejected into aorta and • Atria relax
pulmonary artery
• Figure 10-5 Cardiac cycle. AV, Atrioventricular valves (both tricuspid and mitral). (From Gould B:
Pathophysiology for the health professions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
rhythmically on their own and characteristically are

both voluntary and involuntary responses. Inside the
cavities of the heart is a smooth serous lining called
the endocardium (Figure 10-6). The conduction system
of the heart coordinates the contraction and relaxation
(cardiac cycle) of the heart by initiating impulses and
distributing the impulses throughout the myocardium
(see Figure 10-34). Coronary arteries and a network
of vessels continuously supply cardiac muscle tissue
with oxygen (Figure 10-7).
Cardiovascular Diseases
Endocardium
There are many and varied disorders of the heart and
circulatory system. In some disorders, the rhythm of Myocardium
the heartbeat becomes irregular, may enter tachycar-
dia (become abnormally fast), or may enter bradycar- Visceral pericardium
(epicardium)
dia (become abnormally slow). Disorders of cardiac
rhythm are called arrhythmias or dysrhythmias. Parietal pericardium
Fibrous pericardium
Almost one quarter of all deaths in the United
States are attributed to heart disease, according to the Coronary vessels
CDC. Most of these deaths are caused by coronary Pericardial cavity
artery disease (CAD) and hypertension. Cardiovas- • Figure 10-6 Layers of the heart wall. (From Applegate EJ:
cular disorders, such as angina pectoris, myocardial The anatomy and physiology learning system, ed 4, Phila-
infarction (MI), congestive heart failure (CHF), delphia, 2011, Saunders/Elsevier.)
cardiac arrest, shock, and cardiac tamponade also
Right
coronary artery
Left
coronary artery
Circumflex
branch
Anterior
interventricular Great
branch cardiac vein
Great
cardiac vein Circumflex
branch
Left
coronary
artery
Right
coronary
artery
• Figure 10-7 Coronary arteries.

can result in death. Other diseases of the cardiovas- crushing, and radiating to the arm, neck, or jaw (see
cular system include rheumatic fever, pericarditis, the “Myocardial Infarction” section) and is due to
myocarditis, endocarditis, thromboangiitis obliter- diminished blood flow and lower oxygen saturation.
ans (Buerger disease), Raynaud disease, and vascular Nausea, vomiting, and weakness also can be experi-
(blood vessel) diseases. enced. Changes in the electrocardiogram (ECG) are
Important presenting symptoms that tend to recur often but not always recognized. Many patients may
in patients with cardiovascular disease and need be asymptomatic up until an MI or sudden death
further investigation include: event; this is why noninvasive screening of high-risk
• Chest pain patients is imperative.
• Dyspnea (difficulty in breathing) on exertion
• Tachypnea (rapid breathing) Patient Screening
• Palpitations (rapid fluttering of the heart) Severe chest pain of sudden onset with or without
• Cyanosis (slight blue color) previous diagnosis of angina is considered to be a
• Edema cardiac event and has the potential for being cata-
• Fatigue strophic; therefore the patient should immediately be
• Syncope (fainting) entered into the emergency medical system.
Etiology
Lymphatic and Blood Disorders
Deposits of fat-containing substances called plaque in
See disorders under discussion of specific diseases. the lumen (opening) of the coronary arteries result in
atherosclerosis and subsequent narrowing of the lumen
Coronary Artery Disease of the arteries (Figure 10-8). The myocardium must
have an adequate blood supply to function. The coro-
Description nary arteries supply the cardiac muscle with blood but
CAD is a condition involving the arteries supplying become constricted by atherosclerosis (Figure 10-9).
the myocardium (heart muscle) (see Figure 10-7). The Arteriosclerosis, commonly called “hardening of
arteries become narrowed by atherosclerotic deposits the arteries,” is associated with the elderly and diabet-
over time, causing temporary cardiac ischemia and ics. The arteries eventually lose elasticity and become
eventually MI (or heart attack). hard and narrow, resulting in cardiac ischemia. The
cells in the myocardium gradually weaken and die.
ICD-9-CM Code 414.0 (Requires fifth digit)
Replacement scar tissue forms, interfering with the
ICD-10-CM Code I25.10 (Atherosclerotic
heart disease of native heart’s ability to pump, resulting in heart failure.
coronary artery without People at higher risk for CAD are those who have
angina pectoris) a genetic predisposition to the disease, those older
(I25.10-I25.119 = 5 codes than 40 years of age, men (slightly more than women),
of specificity) postmenopausal women, and Caucasians. Other
Codes for coronary vascular disease are factors contributing to increased risk of the disease
classified by location and type. Refer to the include a history of smoking; residence in an urban
physician’s diagnosis and then to the current society; the presence of hypertension, diabetes, or
editions of the ICD-9-CM and ICD-10-CM coding obesity; and a history of elevated serum cholesterol or
manuals to ensure the greatest specificity.
reduced serum high-density lipoprotein (HDL) levels.
Lack of exercise (a sedentary lifestyle) and stress are
Symptoms and Signs additional risk factors.
Patients are asymptomatic initially, with the first
symptom being the pain of angina pectoris (see the Diagnosis
“Angina Pectoris” section). In advanced disease, the The patient usually does not experience chest pain
severe pain of MI is described as burning, squeezing, from atherosclerosis until the coronary arteries are
Endothelial
damage—
cells and lipid
accumulate
Tunica Atheroma—
intima mass of
fibrofatty
Tunica material,
media cells, and
lipid
Adventitia
A. Fatty streak B. Plaque
Small lumen Total occlusion

of artery
Artery
by thrombus
partially
obstructed
Thrombus
Atheromatous
plaque
C. Formation of thrombus D. Complications

Necrosis or
calcification
weakens wall
3. Embolus
blocks small
artery
2. Embolus flows
along artery
1. Thrombus
breaks off
plaque
E. Embolus
• Figure 10-8 Development of an atheroma leading to arterial occlusion. (From Gould B: Pathophysiol-
ogy for the health professions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
ATHEROSCLEROSIS HEART
Coronary arteries ANGINA PECTORIS
Narrowing of large partial occlusion (Ischemic heart disease)
arteries by cholesterol
plaque and thrombus
Total occlusion MYOCARDIAL
INFARCTION (MI)
BRAIN
Carotid or cerebral arteries TRANSIENT ISCHEMIC
Clear artery partial occlusion ATTACK
Total occlusion CEREBROVASCULAR

ACCIDENT (CVA)
PERIPHERAL ARTERIES
AORTA ANEURYSM — Occlusion
Partial obstruction
— Rupture and
hemorrhage
LEGS
Iliac arteries PERIPHERAL VASCULAR
DISEASE — Gangrene and
amputation
Total obstruction
• Figure 10-9 Possible consequences of atherosclerosis. (From Gould B: Pathophysiology for the health
professions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
about 75% occluded. Collateral circulation often line drug therapy for the prevention of CAD may
develops to supply the tissue with needed oxygen and include angiotensin-converting enzyme (ACE) inhibi-
nutrients (Figure 10-10). An ECG shows ischemia tors, angiotensin receptor blockers (ARBs), calcium
(caused by a lack of blood supply) and possibly channel blockers (CCBs), thiazide diuretics, or vaso-
arrhythmias. Treadmill testing, thallium or Cardiolite dilators. Beta-blockers and anticoagulants are used to
scan, computed tomography (CT) scans, stress echo- prevent blood clots from breaking off and lodging
cardiograms, cardiac catheterization, and angiograms in cerebral arteries. When the blockage is severe
are other tools of cardiac status evaluation used to or does not respond to drug therapy or angioplasty,
detect insufficient oxygen supply and to confirm the coronary artery bypass surgery may be indicated
diagnosis. Electron beam computerized testing, a non- to restore circulation to the affected myocardium
invasive assessment identifying calcium buildup in (Figure 10-12).
arteries, is another means of risk evaluation. Experimental gene therapy uses injections of
deoxyribonucleic acid (DNA) directly into cardiac
Treatment muscle to stimulate new growth of blood vessels; this
Treatment consists of measures to restore adequate is still very preliminary.
blood flow to the myocardium. Vasodilators and other
types of medicines are prescribed. Angioplasty with a Prognosis
balloon or stenting is attempted in some instances to The prognosis varies and depends on the patient’s
open the constricted arteries (Figure 10-11). Claims response to the treatment, whether prescribed drug
of reduction of the plaque buildup with hypolipid- therapy, angioplasty, or coronary bypass surgery. An
emic drugs are being confirmed in some cases. First additional factor affecting the prognosis of smokers is
CHAPTER 10 Diseases and Conditions of the Circulatory System 501.e1
Pulmonary
Superior artery
vena
cava
Right
ventricle
Inferior
vena
cava
Femoral
vein
E10-1 Right-sided cardiac catheterization. (From Ignatavi-

cius: Medical-Surgical Nursing, ed 6, Philadelphia, 2010,
Saunders.)
Collateral circulation
may develop with a
partial obstruction
3. Artery B dilates
1. Partially obstructed artery A
2. Less blood flow here

Superior
vena cava
4. Capillaries from artery B open
and extend to provide collateral
Aorta blood supply
Pulmonary Left atrium

artery
Aortic semilunar valve
Right
atrium Left coronary artery
Right
coronary artery
Left ventricle Branches of left coronary artery
Right
ventricle
Anastomosis is a connection
between branches of two arteries
• Figure 10-10 Collateral circulation of the heart. (From Gould B: Pathophysiology for the health profes-
sions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
the effect of smoking on the coronary arteries and Patients are encouraged to reduce stress and, if they
whether the patient ceases smoking. smoke, to stop or reduce smoking.
Prevention Patient Teaching

Measures to prevent CAD include a diet that is low Give patients information about symptoms of impend-
in salt, fat, and cholesterol, combined with exercise. ing MI, and encourage them to seek immediate
Catheter in Balloon Arterial

narrowed expanded lumen
artery opened
Coronary
occlusion
• Figure 10-11 Angioplasty.
Angina Pectoris
Description
Angina pectoris, chest pain due to ischemia during or
Bypass shortly after exertion, is the result of reduced oxygen
graft
supply to the myocardium.
ICD-9-CM Code 413.9 (Other and unspecified
Bypass angina pectoris)
graft ICD-10-CM I20.8 (Other forms of angina
Code pectoris)
I20.9 (Angina pectoris,
Occlusion unspecified)
(I20.0-I20.9 = 4 codes of
specificity)
Codes for angina pectoris are classified by type.
Refer to the physician’s diagnosis and then to
the current editions of the ICD-9-CM and
ICD-10-CM coding manuals to ensure the
greatest specificity.
• Figure 10-12 Coronary artery bypass.
emergency medical care at the first sign of any related Symptoms and Signs
symptoms. Offer printed information to all patients The patient has a sudden onset of left-sided chest pain
about the prevention or control of CAD and emphasize during or shortly after exertion. The pain may radiate
the importance of a low-fat diet, weight control, exer- to the left arm or back (Figure 10-13). The patient
cise, and cessation of smoking. Encourage follow-up also may experience dyspnea. The pain usually is
cholesterol blood tests. relieved by ceasing the strenuous activity and placing
Treatment
Treatment consists of cessation of the strenuous activ-
ity and the placing of nitroglycerin tablets under the
tongue. Transdermal nitroglycerin helps in preventing
angina. When angina persists after treatment or for
more than 20 minutes, immediate medical attention
is indicated.
Prognosis
The prognosis varies and depends on the extent of the
arterial involvement. When patients can stop the pain
by ceasing strenuous activities and using vasodilating
• Figure 10-13 Common sites of pain in angina pectoris.
medications, the angina usually will diminish or dis-
appear. The patient’s ability to modify his or her life-
nitroglycerin tablets sublingually or using nitroglyc- style may improve the prognosis.
erin spray also sublingually (under the tongue). The
blood pressure may increase during the attack, and Prevention
arrhythmias may occur. Prevention is similar to that recommended for CAD.
Recommendations focus on lifestyle modification,
Patient Screening including appropriate exercise; a diet low in fat, cho-
Patients experiencing symptoms of angina for the first lesterol, and salt; control of hypertension; weight loss;
time require immediate assessment. The sudden onset and smoking cessation. Patients are encouraged to
of chest pain could represent a life-threatening condi- reduce stress.
tion, and acute MI must be ruled out. Those who have
been diagnosed with angina pectoris, and in whom Patient Teaching
cessation of activity and use of vasodilating medica- Patients and families should be given dietary informa-
tions does not provide relief from the pain within 20 tion and suggestions for menu planning within the
minutes, require immediate medical intervention appropriate diet. Emphasize the importance of com-
through the emergency medical system. pliance with prescribed drug therapy. Help the patient
and family locate and contact community services and
Etiology support groups.
Atherosclerosis causes a narrowing of the coronary Patients should be instructed to carry the nitroglyc-
arteries, compromising the blood flow to the myocar- erin tablets with them at all times. Additionally,
dium. Exertion requires increased blood flow to patients and their families should be instructed that
supply more oxygen, but the vessels cannot supply it. the tablets should not be exposed to light or air
Spasms of the coronary arteries also may be a causative and therefore the tablets should be kept in the
factor. Severe prolonged tachycardia, anemias, and original light-resistant bottle with a cap that can be
respiratory disease also can cause cardiac ischemia. tightened.
Diagnosis
Myocardial Infarction
The patient history reveals a previous exertional chest
pain. An ECG taken during the anginal episode may Description
show ischemia; it is important to realize that a normal MI is death of myocardial tissue caused by the devel-
ECG does not preclude the diagnosis of angina. Other opment of ischemia.
diagnostic measures, such as those described for CAD, ICD-9-CM 410 (Acute myocardial
are performed. Code infarction)
ICD-10-CM I21.0 (fifth digit required) (ST

Code elevation [STEMI] myocardial
infarction of anterior wall)
(I21.01-I21.4 = 9 codes of
specificity)
Acute myocardial infarction has many codes
according to episode and location. The code can
include a fifth-digit subclassification according to
episode. Codes for location are as follows: Coronary
ICD-9-CM 410.0 (Of anterolateral wall) occlusion
Code 410.1 (Of other anterior
wall)
410.2 (Of inferolateral wall)
410.3 (Of inferoposterior Myocardial
infarct
wall)
410.4 (Of other inferior wall)
410.5 (Of other lateral wall)
410.6 (True posterior wall • Figure 10-14 Myocardial infarction (MI).
infarction)
410.7 (Subendocardial
infarction) Symptoms and Signs
410.8 (Of other specified
sites) An occlusion of a coronary artery resulting in ischemia
410.9 (Unspecified site) and infarct (death) of the myocardium causes sudden,
ICD-10-CM I21.09 (ST elevation [STEMI] severe substernal or left-sided chest pain (Figure
Code myocardial infarction 10-14). The pain may be crushing, causing a feeling
involving other coronary of massive constriction of the chest, may be burning,
artery of anterior wall) or may just be a vague discomfort. This pain may
I21.19 (ST elevation [STEMI] radiate to the left or right arm, back, or jaw and is not
myocardial infarction relieved by rest or the administration of nitroglycerin.
involving other coronary Irregular heartbeat, dyspnea, and diaphoresis often
artery of inferior wall)
accompany the pain, and the patient usually exhibits
I21.11 (ST elevation [STEMI]
myocardial infarction
denial and experiences severe anxiety, sometimes with
involving right coronary the feeling of impending doom (Figure 10-15). Some
artery) may experience nausea and diaphoresis. MI occasion-
I21.29 (ST elevation [STEMI] ally is clinically silent, especially in diabetics.
myocardial infarction
involving other sites) Patient Screening
I21.4 (Non-ST elevation Early and immediate intervention improves the
[NSTEMI] myocardial chance for survival and minimizes irreversible injury
infarction) to the myocardium. Recent recommendations include
I21.3 (ST elevation [STEMI] calling 911 for entrance into the emergency medical
myocardial infarction of
system and chewing one 5 grain/325 mg aspirin
unspecified site)
Note: The physician must designate the area of
tablet. Emergency intervention must be initiated
the infarction before a code is applied to the immediately to control pain, stabilize heart rhythm,
episode. Refer to the physician’s diagnosis and and minimize damage to the heart muscle. Most
then to the current editions of the ICD-9-CM and deaths caused by an MI result from primary ventricu-
ICD-10-CM coding manuals to ensure the lar fibrillation. Thus immediate ECG monitoring and
greatest specificity. possible defibrillation are of primary concern. The
Left of sternum Mid-chest, Mid-chest

or entire upper chest neck, and jaw and inside arms
(left more than right)
Upper abdomen Chest, neck, Center of lower

jaw, and inside arms neck to both sides
of upper neck and
all of jaw
Inside right arm Between

to below elbow, shoulder blades
shoulder, and inside
left arm to waist (left
side more often than right)
• Figure 10-15 Locations of pain from myocardial infarction (MI). (From Mosby’s dictionary of medicine,
nursing and health professions, ed 7, St Louis, 2010, Mosby/Elsevier.)
American Heart Association (AHA) and the American spasm (Figure 10-16). The pain is caused by ischemia,
Red Cross currently recommend defibrillation train- and if ischemia is not reversed within about 6 hours,
ing for all certified first responders. The latest technol- the cardiac muscle dies. Coronary thrombosis is the
ogy in automated external defibrillators (AEDs) most common cause of MI.
affords auditory instructions to the rescuer, making
the use safe for victim and rescuer. Diagnosis
The diagnosis includes a thorough history, ECG, chest
Etiology radiographic studies, and laboratory tests for cardiac
MI results from insufficient oxygen supply, such as enzyme levels. Changes in enzyme levels indicate the
occurs when a coronary artery is occluded by athero- death of cardiac tissue and include (1) creatine phos-
sclerotic plaque, thrombus, or myocardial muscle phokinase (CPK) and troponin, which are elevated in
Saphenous
vein grafts
Aorta
Right coronary
artery
Circumflex
artery
Left coronary
artery
Anterior Lateral
Left anterior
descending artery
• Figure 10-17 Coronary bypass graft. (From Mosby’s dic-

tionary of medicine, nursing and health professions, ed 7, St
Louis, 2010, Mosby/Elsevier.)
Treatment
Oxygen is administered, and morphine is given for
pain. Aspirin is given as soon as possible to reduce
the risk of additional damage to the heart and tissue
by ischemia. Vasodilation is attempted by nitro-
glycerin drip. Lidocaine or amiodarone given by an
Septal Inferior
intravenous (IV) drip, after a loading bolus, helps to
control arrhythmias. Thrombolytic drugs, including
tissue plasminogen activator (TPA), streptokinase, or
• Figure 10-16 Common locations of myocardial infarction
(MI). (From Lewis SM, Heitkemper MM, Dirksen SR: Medical-
alteplase (Activase) may be administered as soon as
surgical nursing: assessment and management of clinical possible after the diagnosis, unless there are contra-
problems, ed 6, St Louis, 2004, Mosby/Elsevier.) indications. Within the 6-hour window before per-
manent damage, an attempt may be made to open
the occlusion and to restore blood flow to the area
by angioplasty (see Figure 10-11), the administration
the first 6 to 24 hours after MI; (2) lactate dehydro- of thrombolytic drugs, or by coronary artery bypass
genase (LDH), which peaks at 48 hours after MI; and surgery (Figure 10-17). Currently, the standard of care
(3) aspartate aminotransferase (AST). When an eleva- is to try to emergently open the artery with a stent
tion of these enzymes is detected, a study of cardiac (Figure 10-18), preferably within 60 to 90 minutes of
isoenzymes is ordered to confirm the diagnosis. ECG arrival; this has been demonstrated to more effectively
changes in the P-R and QRS complexes and in the decrease heart damage than IV thrombolytic drugs.
ST segment correspond to the ischemic areas. Diag-
nostic confirmation is assisted by elevated cardiac Prognosis
enzyme levels and altered isoenzyme levels identified The prognosis of MI is determined by immediate defi-
through blood tests. brillation for ventricular fibrillation. Late mortality
E10-2 Angiographic images before, after, and late follow-up after placement of a sirolimuseluting stent.
(From Goldman: Goldman’s Cecil Medicine, ed 24, Philadelphia, 2012, Saunders.)
Plaque
Stent
Inflated balloon positioned
expands the stent at lesion
Implanted • Figure 10-19 Photo of an automated external defibrillator

stent (AED). (Courtesy of David Frazier, 2011, in cooperation with
St Johns County Sheriff’s Office, St Augustine, FL.)
Cardiac Arrest
• Figure 10-18 Coronary artery stent. (From Mosby’s dic- Description
tionary of medicine, nursing and health professions, ed 7, St
Louis, 2010, Mosby/Elsevier.) Cardiac arrest is the sudden, unexpected cessation of
cardiac activity.
ICD-9-CM Code 427.5
ICD-10-CM Code I46.9 (Cardiac arrest, cause
unspecified)
depends on the extent of damage to the heart muscle (I46.2-I46.9 = 3 codes of
and the occurrence of complications. Most late specificity)
cardiac death is sudden, caused by the onset of a fatal
arrhythmia. Symptoms and Signs
The patient is unresponsive, with no respiratory effort
Prevention and no palpable pulse.
The prevention is similar to that recommended for
CAD. Recommendations focus on lifestyle modifica- Patient Screening
tion, including appropriate exercise; a diet low in fat, Cardiac arrest is a true life-threatening emergency.
cholesterol, and salt; control of hypertension; weight Immediate intervention with instantaneous initiation
loss; and smoking cessation. Patients are encouraged of cardiopulmonary resuscitation (CPR) and defibril-
to reduce stress. Those surviving an MI are urged to lation by means of an AED may successfully restore
take a daily aspirin dose and a beta-blocker or ACE contraction of the heart (Figure 10-19). The AHA or
inhibitor for life. Lipid-lowering medications are also American Red Cross protocol for caregivers in the
recommended. field requires immediate contact of the emergency
medical system by calling 911. Inpatient facilities use
Patient Teaching the “Code Blue” message to alert personnel.
Give patients and families dietary information and
suggestions for menu planning within the appropriate Etiology
diet. Emphasize the importance of compliance with Cardiac arrest results from anoxia (absence of oxygen
prescribed drug therapy and cardiac rehabilitation. to the tissue) or interruption of the electrical stimuli
Help the patient and family find and contact com- to the heart. It can be caused by respiratory arrest,
munity services and support groups. arrhythmia, or MI. Electrocution, drowning, severe
trauma, massive hemorrhage, or drug overdose also lifestyle modifications can reduce risk. One cannot
can cause cardiac arrest. predict near-drowning or electrical shock accidents,
but the prudent individual will try to avoid situations
Diagnosis that carry this risk. Recently, in high-risk patients with
The diagnosis is based on the absence of respiratory a history of abnormal heart rhythms or weak heart
effort and lack of palpable pulse. The ECG shows muscle, the implanting of defibrillators has been
ventricular fibrillation or asystole. shown to decrease the risk of sudden cardiac death.
Treatment Patient Teaching

CPR must be instituted within 4 to 6 minutes of the Encourage all possible candidates for CPR training to
cardiac arrest. Until recently, cardiac defibrillation was become certified. Help families of patients who do not
only attempted by trained, advanced life-support per- survive cardiac arrest to find and contact support
sonnel. AEDs are now available for use by anyone groups in the community. Emphasize safety guidelines
who observes a cardiac arrest. The latest technology to prevent drowning and electrical shock.
implemented in these devices talks the rescuer through Encourage survivors of cardiac arrest to comply
the defibrillation process (see Figure 10-19). The with the prescribed regimen of activities and drug
rescuer should be familiar with the device he/she therapy. Survivors also may need help in finding and
is attempting to use. Ambulance or other medical contacting support groups for survivors of cardiac
personnel administer cardiac drugs, including epi- arrest.
nephrine (Adrenalin) and isoproterenol (Isuprel) or
dobutamine to stimulate the heart. Antiarrhythmic
drugs, including lidocaine and amiodarone, also may
be administered. ALERT!
The American Heart Association (AHA) has
Prognosis updated protocol for cardiopulmonary resuscitation
The prognosis varies depending on the length of time (CPR) on an adult cardiac arrest victim. Upon
the individual has been in cardiac arrest. The earlier observing an individual in what is suspected as
cardiac arrest, the first step is to call out for help
in the event that CPR and defibrillation are instituted,
and activate the emergency medical service (EMS)
the greater possibility there is for survival. Within 1 system (911). The next step is to immediately start
to 2 minutes after cessation of cardiac activity, respira- giving 30 rapid deep chest compressions (30 in 18
tory efforts cease. At 4 to 6 minutes after the cessation seconds). The rescuer may now give two rapid
of cardiac activity, brain cells begin to die. At 10 rescue breaths followed by resumption of rapid chest
compressions. The traditional ABCs of CPR are now
minutes after the cardiac activity has ceased with no
referred to as CAB (compressions, airway, and
intervention, the brain dies and death is inevitable. breathing).
Many public venues and emergency vehicles now have See the Enrichment box about Therapeutic
portable defibrillators to allow more rapid resumption Hypothermia.
of cardiac function. Courtesy of George L. Schiffman, MD, FCCP, Mission Internal Medical
Successful resuscitation depends on immediate and Group, Mission Viejo, CA, and AHA updated protocol for post arrest
complete intervention. Other factors that affect the treatment.
final outcome of the event include the general health

and age of the patient and the cause of the arrest.
Successful interventions in cold water near-drowning Hypertensive Heart Disease
and electrical shock have been recorded.
Hypertensive heart disease is the result of chronically
Prevention elevated pressure throughout the vascular system. Ath-
Prevention of the catastrophic event of cardiac arrest erosclerosis, arteriosclerosis, renal disease, and any
is problematic. However, as in CAD and MI, the same condition that creates increased vascular pressure
ENRICHMENT few, if any, symptoms until permanent damage has

occurred.
Therapeutic Hypothermia
ICD-9-CM Code 401 (Requires fourth digit)
Patients following cardiac arrest and ICD-10-CM Code I10 (Essential [primary]
cardiopulmonary resuscitation (CPR) often have a hypertension)
bad outcome, either death or severe brain damage. Essential hypertension is listed in several ways
However, many have heard reports of people who under hypertensive heart disease. Other
fell into lakes in cold climates being resuscitated mentions of hypertension are listed under
after some time and returning to normal lives. hypertensive heart disease, hypertensive renal
Recent studies have shown that cooling a select disease, hypertensive heart and renal disease,
group of patients immediately following cardiac and secondary hypertension. Refer to the
arrest can improve neurologic outcome and quite
physician’s diagnosis and then to the current
possibly decrease mortality. This is referred to as
editions of the ICD-9-CM and ICD-10-CM coding
therapeutic hypothermia. Patients whose mental
status remains impaired are rapidly cooled to 91° F manuals to ensure the greatest specificity.
(32° C to 33° C) immediately following cardiac arrest.
This can be performed using a variety of devices, Symptoms and Signs
some as simple as cooling blankets and ice, to
cardiopulmonary bypass. This can be quite The patient may experience headaches, epistaxis, light-
uncomfortable for patients, because the body fights headedness, or syncope, although there usually are no
to maintain normal temperature, primarily by symptoms. The hypertension generally is detected
shivering. Therefore patients are heavily sedated and when blood pressure is taken during a physical exami-
maintained on mechanical ventilation. Patients must
be monitored quite closely for shifts in many of their
nation or screening process. Hypertension is more
electrolytes and the possibility of seizures. This is common with increased age in all groups. If hyperten-
continued for 18 to 24 hours. Then the process of sion is accompanied by hyperlipidemia, it may lead to
rewarming is begun. This is perhaps the most atherosclerosis.
critical time of this therapy. Rapid rewarming can be
disastrous and must be performed with the utmost Patient Screening
care. Several sophisticated devices have been
developed to slowly rewarm patients by less than a Many patients with essential hypertension have no
degree an hour (e.g., external devices, such as the indication of the condition. They may seek an appoint-
Artic Sun, or internal devices, such as the Inner cool ment for a headache and should be scheduled for the
catheter). As this therapy becomes more next available appointment. Others may have recently
commonplace following cardiac arrest, mortality will
be reduced and better neurologic outcomes are
had a blood pressure reading at a health screening or
likely to result. other event and have been advised to see their physi-
cian to report the elevated reading. These patients also
(Courtesy of George L. Schiffman, MD, FCCP, Mission Internal
Medical Group, Mission Viejo, CA.) should be scheduled for the next available appoint-
ment, preferably on the day of or the day after the
call. Likewise, patients with a history of essential
hypertension requesting an appointment for any
hypertensive-related symptoms should be scheduled
cause the heart to work harder as it pumps against the that day or the next day.
increased resistance.
Etiology
The etiology is unknown, but many factors are thought
Essential Hypertension
to contribute to the condition. Stress is considered a
Description major factor in hypertension. Age, heredity, smoking,
Essential, or primary, hypertension, a condition of obesity, sedentary lifestyle, poor dietary habits, and
abnormally high blood pressure in the arterial system, hyperactive personality or type A personality are possible
has an insidious onset, with the patient exhibiting causative factors in essential hypertension (Figure 10-20).
Systemic
vasoconstriction
Nephrosclerosis
Decreased blood flow to kidney
Diabetic nephropathy
Increased renin secretion
Systemic Aldosterone
vasoconstriction secretion
Increased Increased
peripheral blood
resistance volume
Damage to
renal blood vessels Increased
blood pressure
Increased work for the heart

causing left-sided CHF
Damage to cerebral Damage to

arteries causing stroke retinal blood vessels
causing blindness
Damage to arterial walls
leading to atherosclerosis
• Figure 10-20 Development of hypertension. (From Gould B: Pathophysiology for the health profes-
sions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
to increase renal blood flow), ARBs, vasodilators (to

Diagnosis dilate vessels), and CCBs (to slow the heartbeat, to
Elevated blood pressure readings are the first indica- reduce conduction irritability, and to dilate vessels).
tion of hypertension. A systolic reading of greater than These drugs may be prescribed singly or in combina-
140 mm Hg and a diastolic reading of greater than tion. Choosing the best first medication is a continu-
90 mm Hg indicate hypertension. Recent guidelines ous debate. The World Health Agency, the Cochrane
have established that systolic readings of 120 to collaboration and the U.S. guidelines promote a low
140 mm Hg indicate borderline hypertension. The dose thiazide diuretic as first line treatment. The UK
diagnosis is based on a series of blood pressure read- recommends CCBs for patients over 55 and ACE
ings in which elevated values are obtained several inhibitors for younger people. A regimen of drug
times. A careful, complete medical history, physical therapy is designed to fit each patient’s needs and
examination, and laboratory evaluation should be per- response. Additional modes of therapy include limi-
formed before diagnosis is confirmed and therapy is tation of sodium intake, dietary management, weight
initiated. reduction, exercise, reduction of stressful situations,
and cessation of smoking.
Treatment
Drug therapy used in the treatment of hyperten- Prognosis
sion includes diuretics (to reduce circulating blood The prognosis varies and depends on the patient’s
volume), ACE inhibitors (to produce vasodilation and response to prescribed drug therapy and lifestyle
modifications. A reduction of blood pressure by as little Some may report having been advised to contact their
as 5% may decrease the risk of stroke by 30% or more. doctor for an elevated blood pressure reading. Others
may complain of severe headache. Previously diag-
Prevention nosed essential hypertensive patients may complain of
Because the etiology is unknown, one cannot always the sudden onset of a severe headache, blurred vision,
prevent essential hypertension. The patient may be and dyspnea. These patients are at risk for a cerebro-
able to alter contributing factors by reducing stress vascular accident (CVA) and require prompt assess-
levels, reducing sodium intake, controlling weight, ment and intervention.
exercising and stopping smoking. Altering the indi-
vidual’s underlying personality, which plays a part in Etiology
the condition, is difficult. The etiology of this severe form of essential hyperten-
sion is unknown, although extreme stress is thought
Patient Teaching to be a contributing factor.
Teaching the patient that this condition is not cured
but only controlled by drug therapy is essential. Edu- Diagnosis
cation reinforces the need to monitor the blood pres- Notable blood pressure elevation is considered malig-
sure on a regular basis and the need to continue drug nant hypertension. In severe cases, the systolic pres-
therapy for life. When available, use computer-based sure reading may be greater than 200 mm Hg and the
health education to teach patients about home moni- diastolic pressure reading greater than 120 mm Hg.
toring of blood pressure.
Treatment
Aggressive intervention is indicated in severe malig-
Malignant Hypertension
nant hypertension. IV vasodilators, such as diazox-
Description ide (Hyperstat) and sodium nitroprusside (Nipride),
Malignant hypertension, a life-threatening condition, should be administered. After the condition is under
is a severe form of hypertension. control, blood pressure should be monitored on a
regular basis and drug therapy continued for life.
ICD-9-CM Code 401.0
ICD-10-CM Code I10 (Essential [primary] Prognosis
hypertension)
Malignant hypertension is listed in several ways The prognosis varies and depends on the patient’s
under hypertensive heart disease. Other mentions response to drug therapy. These patients are at risk for
of malignant hypertension are listed under a CVA, or stroke, and irreversible renal damage. When
hypertensive heart disease, hypertensive renal drug therapy is unsuccessful, the patient is likely to
disease, hypertensive heart and renal disease, and succumb to the condition after a CVA.
secondary hypertension. Refer to the physician’s
diagnosis and then to the current editions of the Prevention
ICD-9-CM and ICD-10-CM coding manuals to With etiology being unknown, preventing this condi-
ensure the greatest specificity of pathology.
tion is difficult. Those who have been diagnosed as
hypertensive, however, should comply with drug
Symptoms and Signs therapy and reduce their stress. The preventive mea-
Severe headache, blurred vision, and dyspnea are sures suggested for essential hypertension also apply
symptoms that suggest the condition. The symptoms to malignant hypertension.
may have sudden onset.
Patient Teaching
Patient Screening Emphasize the importance of complying with drug
As with essential hypertension, many people with therapy to patients and their families. Encourage
malignant hypertension are unaware of the condition. patients to modify lifestyles to reduce stress in their
lives. Give patients and families dietary information

about low-fat, low-cholesterol, and low-sodium diets
Pulmonary Distended
and management. Advise overweight patients about congestion neck veins
the importance of weight reduction and exercise.
Encourage discussion concerning the dangers of Enlarged
malignant hypertension. Hepatic heart
congestion
Lymphatic and Blood Disorders

See disorders under discussion of specific diseases.
Congestive Heart Failure

Description
CHF is an acute or chronic inability of the heart to
pump enough blood throughout the body to meet the
demands of homeostasis.
Abdominal
ICD-9-CM Code 428.0 (Unspecified) edema
ICD-10-CM Code I50.9 (Heart failure,
unspecified)
(150.1-I50.9 = 14
codes of specificity)
Refer to the physician’s diagnosis and then to the Edema of
current editions of the ICD-9-CM and ICD-10-CM legs and feet
coding manuals to ensure the greatest specificity.
Symptoms and Signs

CHF usually has an insidious onset with the patient • Figure 10-21 Signs of congestive heart failure (CHF).
experiencing gradually increasing dyspnea. Cardiac
and respiratory rates increase, and the patient becomes
anxious. As the condition progresses, the neck veins MI (Figure 10-22). Some causes of chronic CHF are
distend and edema is noted in the ankles. When hypertension, CAD, chronic obstructive pulmonary
the right side of the heart fails, the liver and spleen disease (COPD), cardiac valve damage, arrhythmias
enlarge and the peripheral edema is more prominent. (dysrhythmias), and cardiomyopathy.
Left-sided CHF causes increased pulmonary conges-
tion and more pronounced respiratory difficulties Diagnosis
(Figure 10-21). The diagnosis is made after a thorough history and
physical examination. Breath sounds are diminished,
Patient Screening and radiographic film indicates the presence of fluid
Patients reporting unexplained chest discomfort, in the lungs (Figure 10-23). An ECG is used to dis-
shortness of breath, or swelling of limbs require cover the underlying causes. An echocardiogram helps
prompt medical assessment. in evaluating cardiac chamber size, ventricular func-
tion, and disease of the myocardium, valves, cardiac
Etiology structures (walls, septum, and papillary muscles), and
Underlying conditions can compromise the pumping pericardium (the covering of the heart) (Figure 10-24).
action of the heart, resulting in heart failure and inad- Catheterization can be used to monitor the pressures
equate perfusion. A common cause of acute CHF is in the circulation.
EXAMPLE LOSS OF
MYOCARDIAL INFARCTION HEART MUSCLE
LEFT VENTRICLE
DECREASED CARDIAC OUTPUT

ACTIVATE COMPENSATION MECHANISMS
TREATMENT TO
BREAK CYCLE STIMULATES SNS, LEADING TO
Vasoconstriction and increased
Alpha-adrenergic blockers
Vasodilators resistance for left ventricle
Calcium blockers
Beta blockers Increased heart rate and force —
Digoxin increased work for heart
STIMULATES RENIN SECRETION, LEADING TO
ACE inhibitors Activation of angiotensin–vasoconstriction
Diuretics Stimulation of aldosterone secretion

Increased Na+ and water retention
Increased blood volume
Increased work for heart

COMPENSATIONS MAINTAIN CARDIAC
OUTPUT FOR A TIME
HEART MUSCLE (LEFT VENTRICLE) WEAKENS
LEFT VENTRICLE DOES NOT FULLY EMPTY

DECREASED CARDIAC OUTPUT
LEFT- BLOOD BACKS UP IN PULMONARY CIRCULATION
SIDED
CHF
PULMONARY CONGESTION
INCREASED RESISTANCE FOR RIGHT VENTRICLE
RIGHT VENTRICLE WEAKENS AND CANNOT FULLY EMPTY
RIGHT- BLOOD BACKS UP IN SYSTEMIC CIRCULATION

SIDED
CHF
EDEMA IN LEGS AND DIGESTIVE SYSTEM
• Figure 10-22 Course of congestive heart failure (CHF). ACE, Angiotensin-converting enzyme; CHF,
congestive heart failure; SNS. (From Gould B: Pathophysiology for the health professions, ed 3, Philadel-
phia, 2006, Saunders/Elsevier.)
Patient Teaching
A primary goal is promoting good patient compliance
with the medical treatment plan. Explain that many
patients experience a quick improvement in symp-
toms when they take medications as prescribed, follow
dietary instructions, and modify activities to allow
required rest and to avoid fatigue. The use of diuretics
requires patients to monitor their weight on a daily
basis.
Cor Pulmonale
• Figure 10-23 Radiograph: Congestive heart failure (CHF). Description
(From Long BW, Frank ED, & Ehrlich RA: Radiography essen-
tials for limited practice, ed 4, St Louis, 2013, Elsevier.) Cor pulmonale, also known as right-sided heart disease,
results in enlargement of the right ventricle as a
sequela to primary lung disease.
Treatment ICD-9-CM Code 416.9 (Chronic, NOS)

ICD-10-CM Code I27.81 (Cor pulmonale
Treatment is directed at reducing the workload of the [chronic])
heart and increasing its efficiency. ACE inhibitors and I27.9 (Pulmonary heart
ARBs recently were approved for use in the treatment disease, unspecified)
of CHF, mainly to increase blood flow. Diuretics help (I27.0-I27.9 = 7 codes
to reduce the volume of fluid in the body, and vaso- of specificity)
dilators help to reduce vascular pressure. Aldosterone
antagonists (eplerenone or spironolactone) may be
used in combination with other drug therapies. Symptoms and Signs
Digoxin may also be prescribed in specific cases to Cor pulmonale causes the patient to experience
strengthen and slow the heartbeat. Intake of fluid and dyspnea, distended neck veins, and edema of the
sodium is restricted. Some patients with severe CHF extremities. The liver is enlarged and tender.
may require special pacemakers, defibrillators, or in
extreme cases, a heart transplant. Patient Screening
Patients reporting unexplained chest discomfort,
Prognosis shortness of breath, or swelling of limbs require
The prognosis varies. Acute CHF usually responds prompt medical assessment.
well to medical interventions and therefore has a posi-
tive outcome. The patient with chronic CHF is Etiology
vulnerable to major organ impairment and resulting Right-sided heart failure is an outcome of acute or
complications. chronic pulmonary disease and pulmonary hyperten-
sion. The diseased pulmonary blood vessels impair
Prevention the flow of blood to pulmonary tissue. The increased
Public education about the contributors to heart pulmonary blood pressure increases the workload
health and control of blood pressure continue to of the right side of the heart, causing the right
produce positive statistical results, especially in the ventricle to hypertrophy and thus to become less effec-
male population. Early medical intervention for CHF tive in pumping blood to the lungs. Chronic con-
is important to prevent multiple organ complications. ditions causing cor pulmonale include emphysema
4. Backup of blood into

pulmonary vein
5. High pressure in
pulmonary capillaries
leads to pulmonary
congestion or edema
Lung
L 2. Decreased cardiac output

R to system
1. Left ventricle
weakens and 3. Decreased renal blood flow
cannot empty stimulates renin-angiotensin
and aldosterone secretion
Kidney
Left-sided congestive heart failure Means less

blood here
6. Very high venous pressure

causes distended neck vein
and cerebral edema
Lung
4. Backup
of blood
into systemic
circulation
(venae cavae)
5. Increased
venous pressure 2. Decreased cardiac output
results in edema R to system
in legs and liver
and abdominal
organs 1. Right ventricle
weakens and Kidney
cannot empty
3. Decreased renal blood flow

stimulates renin-angiotensin
and aldosterone secretion
Right-sided congestive heart failure

• Figure 10-24 Effects of congestive heart failure (CHF). (From Gould B: Pathophysiology for the health
professions, ed 2, Philadelphia, 2002, Saunders/Elsevier.)
and fibrotic pulmonary lesions, and the primary Pulmonary Edema

acute causative factor is pulmonary emboli. Chronic
hypoxemia stimulates the bone marrow in an adap- Description
tive response to produce an increased number of red Pulmonary edema is a condition of fluid shift into the
blood cells (RBCs) to carry additional oxygen. This extravascular spaces of the lungs.
condition of abnormally high levels of RBCs (polycy-
ICD-9-CM 518.4 (Acute pulmonary edema
themia) increases the blood viscosity.
Code NOS)
428.1 (Acute with mention of
Diagnosis
heart disease or failure)
The diagnosis is based on a history of pulmonary 514 (Chronic or unspecified)
disease and hypoxia. The patient’s respiratory and ICD-10-CM J81.0 (Acute pulmonary edema)
cardiac status is assessed for neck vein distention Code (J81.0-J81.1 = 2 codes of
and peripheral edema. Radiographic chest studies and specificity)
echocardiography reveal pulmonary congestion and I50.1 (Left ventricular failure)
right-sided heart enlargement, and the ECG fre- J18.2 (Hypostatic pneumonia,
quently shows arrhythmias. If polycythemia is present, unspecified organism)
(J18.0-J18.9 = 5 codes of
the RBC count is elevated.
specificity)
Treatment J81.1 (Chronic pulmonary edema)
Treatment entails relieving the causative factors in the the current editions of the ICD-9-CM and
pulmonary system and reducing hypoxemia. Bron- ICD-10-CM coding manuals to ensure the
chodilators are administered. Supplemental oxygen greatest specificity.
provides additional comfort to the patient. Bed rest is
encouraged, and digitalis preparations are adminis- Symptoms and Signs
tered to strengthen and slow the heartbeat. Diuretics Pulmonary edema causes patients to experience
are prescribed when edema is present. Anticoagulants dyspnea and coughing, orthopnea, a condition in
are given to avoid the risk of thromboembolisms. which breathing becomes easier in an upright standing
Phlebotomy, surgical puncture of a vein to withdraw or sitting position, increased cardiac and respiratory
blood, may be used when polycythemia is a problem. rates, and often bloody, frothy sputum. Blood pressure
A low-salt diet is encouraged. may fall, and the skin becomes cold and clammy. The
symptoms often occur at night after the patient lies
Prognosis down.
The outcome depends on the patient’s response to
treatment of the many possible disorders that predis- Patient Screening
pose to cor pulmonale. Patients complaining of shortness of breath, typically
when lying down, should be told to seek emergency
Prevention care.
No direct prevention of this condition is known.
Many of the predisposing factors have no preventive Etiology
measures to be taken. Pulmonary edema is caused by left-sided heart failure,
mitral valve disease, pulmonary embolus, systemic
Patient Teaching hypertension, arrhythmias, and renal failure. Head
Emphasize the instructions given by the physician. trauma, drug overdose, and exposure to high altitudes
Explain the dosage schedule, and encourage compli- are other causes. Excessive fluid accumulates in the
ance with drug therapy. Give patients information on pulmonary tissue and air spaces of the lungs. The
possible side effects. Advise patients to report adverse pulmonary circulation is overloaded with an excessive
effects from medications. volume of blood (Figure 10-25).
Cyanosis
Elevated jugular
venous pressure
Pleural effusion
HEART
Decreased blood flow LUNG
to coronary arteries Increased transudation Lung edema
Increased congestion
Decreased oxygen
supply to heart Decreased compliance Dyspnea
Decreased ventilation
LIVER
Chronic venous congestion Decreased oxygen uptake Cyanosis
Portal hypertension
Splenomegaly
KIDNEY Ascites
Decreased glomerular filtration
Increased Na+ reabsorption
Increased water retention
Edema
Low urine
output
(oliguria)
Lungs
Right Left
heart heart
Edema
Liver
Kidney
Spleen
Ascites and edema
• Figure 10-25 Chronic passive congestion. Left heart failure leads to pulmonary edema. Right ven-
tricular failure causes peripheral edema that is most prominent in the lower extremities. (From Damjanov
I: Pathology for the health-related professions, ed 4, St Louis, 2011, Saunders/Elsevier.)

Diagnosis ICD-10-CM Code I42.3 (Endomyocardial
The clinical picture of dyspnea, orthopnea, and [eosinophilic] disease)
bloody, frothy sputum leads to further investigation. Cardiomyopathy is coded according to type.
Breath sounds are diminished, with the presence of
rales, rhonchi, and wheezing. Arterial blood gas mea-
surement shows reduced oxygen saturation, increased greatest specificity.
carbon dioxide retention, increased bicarbonate levels,
and decreased pH of the blood. Radiographic chest
films show increased opacity of the pulmonary tissue, Symptoms and Signs
an enlarged heart, and prominent pulmonary vessels. Cardiomyopathy causes the patient to experience
symptoms of CHF, including dyspnea, fatigue, tachy-
Treatment cardia, palpitations, and occasionally chest pain.
The patient is placed in the Fowler position (sitting), Peripheral edema and hepatic congestion also may be
and oxygen therapy is administered. Drug therapy present. Syncope and cardiac murmurs may occur.
includes diuretics to improve fluid excretion; IV The symptoms and signs vary with the type and cause
vasodilators, such as nitroglycerine or nitroprusside; of this condition.
morphine sulfate to induce venous dilation; and β2-
adrenergic drugs to dilate bronchioles and to control Patient Screening
bronchial spasms. Severe cases may require mechani- Patients reporting dyspnea, fatigue, tachycardia, pal-
cal ventilation. pitations, and occasional chest pain require prompt
assessment by a physician.
Prognosis
Pulmonary edema is a life-threatening condition and Etiology
is considered a medical emergency. The prognosis Primary causes are mostly unknown. Cardiomyopa-
varies depending on the severity of the pulmonary thies are divided into three groups: dilated, hypertro-
edema and the patient’s response to intervention. phic, and restrictive. Dilated cardiomyopathy can be
the result of chronic alcoholism, an autoimmune
Prevention process, or viral infections. Regardless of the cause,
Certain causes are controlled by prevention of heart dilated cardiomyopathies result in a diffuse degenera-
disease and avoidance of risk factors, such as drug tion of the myocardial fibers. This is followed by a
overdose. decrease in contractile effort.
Hypertrophic cardiomyopathies are thought to
Patient Teaching be genetic and are considered idiopathic. The left
Encourage compliance with drug therapy and ventricular wall hypertrophies, as does the interven-
follow-up care. Patients taking diuretics are instructed tricular septum, resulting in a small and elongated
about the importance of monitoring weight on a daily left ventricle and possible obstruction of the aortic
basis. valve.
Restrictive cardiomyopathy is a rare condition
Cardiomyopathy occurring when any infiltrative process of the heart
causes fibrosis and thickening of the myocardium
Description resulting in the walls of the ventricles becoming stiff-
Cardiomyopathy is a noninflammatory disease of ened. They are usually normal in size or only slightly
the cardiac muscle resulting in enlargement of thickened. The stiff muscle that does not relax nor-
the myocardium and ventricular dysfunction. Refer mally results in the chamber not filling properly with
to Chapter 2 for discussion about Hypertrophic blood. Thus it is harder for the ventricles to fill with
Cardiomyopathy. adequate blood. The rigidity of the walls does not
LA LA
Ao Ao
LV
LV
Normal Dilated
cardiomyopathy
LA LA
Ao Ao
LV LV
Hypertrophic Restrictive
cardiomyopathy cardiomyopathy
E10-3 Cardiomyopathies. (From Kumar: Robbins Basic Pathology, ed 9, Philadelphia, 2013,
Saunders.)
permit the ventricular muscles to pump correctly

resulting in reduced amounts of blood being pumped Patient Teaching
from the ventricles. The inability of the heart to pump Patient teaching focuses on improving the patient’s
blood properly may lead to heart failure. compliance with the medical treatment plan. Patients
are encouraged to avoid alcohol and to limit salt
Diagnosis intake. Some patients benefit from support groups for
The diagnosis includes a thorough patient history and people living with chronic disease.
a complete physical examination. Cardiomegaly at
various stages is present, along with assorted cardiac Pericarditis
murmurs. The radiographic chest film confirms the
presence of cardiomegaly, and the ECG reveals rate Description
and rhythm abnormalities. Echocardiograms and Pericarditis is an acute or chronic inflammation of the
cardiac catheterization may help to identify the type pericardium (serosa), the sac enclosing and protecting
of cardiomyopathy and the extent of the condition. A the heart (Figure 10-26).
biopsy may be required.
ICD-9-CM Code 423.9 (Unspecified)
Treatment ICD-10-CM Code I31.9 (Disease of
pericardium, unspecified)
Treatment is determined by the type of cardiomyopa- (I30.0-I32 = 12 codes of
thy. Therapy for dilated cardiomyopathies is aimed specificity)
at appropriate control of the CHF by means of the Pericarditis is coded by type. Refer to the
measures previously mentioned for treatment of CHF. physician’s diagnosis and then to the current
Antiarrhythmic agents, digitalis, and anticoagulant editions of the ICD-9-CM and ICD-10-CM coding
drugs are prescribed. Activities are limited, with some manuals to ensure the greatest specificity.
patients restricted to bed rest. Treatment of hyper-
trophic cardiomyopathies also is aimed at reducing
the workload of the heart. Beta-adrenergic blockers,
such as propranolol hydrochloride (Inderal), reduce
the myocardial contractility, the heart rate, and the
conductivity, thus preventing arrhythmias. CCBs are
prescribed to help reduce the blood pressure and relax
the heart muscle. ACE inhibitors are used to help
relax blood vessels and reduce the heart’s work-
load. Treatment of restrictive cardiomyopathies also
includes reducing the workload of the heart. The
changes in the cardiac muscle caused by the infil-
trates are irreversible, making the prognosis for these
patients poor.
Prognosis
Medication improves the survival rate of these patients.
Some conditions can be fatal, with the only hope for
survival being heart transplantation.
Prevention
In many cases, these conditions are idiopathic. Preven- • Figure 10-26 Pericarditis. (From Damjanov I: Pathology
tion is nonspecific depending on the original underly- for the health-related professions, ed 4, St Louis, 2011,
ing causative factor. Saunders/Elsevier.)
Symptoms and Signs Etiology

The space between the outer parietal layer of pericar- Pericarditis is idiopathic or a consequence of inflam-
dium and the inner visceral layer of epicardium (heart mation or infection elsewhere in the body. Other
wall) normally is filled with a small amount of thin, causative agents are viruses, bacteria (producing a sup-
lubricating serous fluid (see Figure 10-6). When blood purative pericarditis), trauma, rheumatic fever, and
or inflammatory exudate is released into the pericar- malignant neoplastic disease. The condition may
dial sac, or pericardial space, friction and irritation occur secondary to MI. Acute inflammation of the
between the layers result in pericarditis. Associated pericardium can cause adhesions (scarring) to form
manifestations include fever, malaise, chest pain that between the pericardium and the heart, or it can cause
fluctuates with inspiration or heartbeat, dyspnea, and a loss of elasticity, producing a constrictive pericardi-
chills. The patient may feel anxious and report a tis. Conversely, chronic pericarditis can incite fibrous
“pounding heart.” A detectable friction rub, or grating calcification of the visceral membrane, which comes
sound, in phase with the heartbeat can be heard in direct contact with the myocardium. A scarred and
on auscultation with a stethoscope. Tachycardia may rigid pericardium interferes with the heart’s ability to
be present. Pericarditis can occur in different forms. contract normally, with a subsequent drop in cardiac
It can be a benign or isolated process or can be second- output (Figure 10-27).
ary to infection elsewhere in the body, so the clinical
signs vary. Diagnosis
Blood studies may lead to the identification of a caus-
Patient Screening ative organism. They also may reveal elevated white
Patients complaining of chest pain are advised to seek blood cell (WBC) count, erythrocyte sedimentation
immediate medical attention. rate (ESR), and cardiac enzyme levels. Changes are
3. Backup into
systemic
circulation 5. Decreased
output to body
Aorta
4. Decreased
blood flow
to lungs
Pericardium
Myocardium
2. Heart cannot
expand to fill Pericardial
cavity
1. Fluid around heart compresses heart wall
• Figure 10-27 Effects of pericardial effusion. (From Gould B: Pathophysiology for the health professions,
ed 3, Philadelphia, 2006, Saunders/Elsevier.)
noted on an ECG. An echocardiogram may confirm age. The patient may report palpitations, fatigue, and
the presence of pericardial fluid and reveal a thickened dyspnea. Physical examination may reveal fever,
pericardium. In constrictive pericarditis, cardiac cath- arrhythmia, and tenderness in the chest.
eterization shows elevated pressures in the cardiac
chambers. Patient Screening
Patients complaining of dyspnea and palpitations are
Treatment advised to seek immediate medical attention.
Treatment is directed at managing the underlying sys-
temic disease and at reducing inflammation and pain. Etiology
Therapy for infectious pericarditis requires antibiotic Myocarditis is frequently a viral, bacterial, fungal, or
drugs and possibly surgical drainage or aspiration. protozoal infection or a complication of other dis-
Complete bed rest and the administration of analge- eases, such as influenza, diphtheria, mumps, and most
sics, antipyretics, and nonsteroidal antiinflammatory significantly, rheumatic fever; it is occasionally idio-
drugs (NSAIDs) are prescribed. Corticosteroids may pathic. It also may be associated with a MI. Exposure
be prescribed. to certain toxic agents through lithium use, chronic
use of cocaine, chronic alcoholism, radiation, and
Prognosis chemical poisoning, can cause inflammation of the
Acute pericarditis usually resolves with complete myocardium. In addition, it may be a complication of
recovery. Extensive adhesions or calcification resulting a collagen disease.
from chronic pericarditis may necessitate resection of
the pericardium. Diagnosis
Diagnostic findings may include an elevated WBC
Prevention count, increased ESR, elevated cardiac enzyme levels,
Prompt treatment of infections is a preventive measure ventricular enlargement noted on radiographic chest
for pericarditis. film, and an abnormal ECG. Myocardial biopsy con-
firms the inflammation of cardiac muscle tissue and
Patient Teaching may identify the cause.
Encourage the patient to seek prompt treatment for
infections. Emphasize the importance of completing Treatment
the antibiotic regimen. When infection is the underlying cause, appropriate
antiinfective agents are given. The patient is advised
to rest and to reduce the heart’s workload. Medica-
Myocarditis tions, such as quinidine procainamide, digoxin, and
diuretics, may be required to stabilize arrhythmia.
Description Analgesics, antiinflammatory agents, ACE inhibitors,
Myocarditis is inflammation of the muscular walls of and oxygen are also prescribed.
the heart.
Prognosis
ICD-9-CM Code 429.0 (Unspecified)
The prognosis for complete recovery is favorable,
ICD-10-CM Code I51.4 (Myocarditis,
unspecified) unless the condition is chronic and causes damage to
the cardiac muscle.
Symptoms and Signs Prevention

Myocarditis involves damage to the myocardium by Depending on the underlying cause, prevention is not
pathogenic invasion or toxic insult. The condition always possible. In some cases, early treatment of
may be acute or chronic, may involve a small part of infections with a complete course of antimicrobial
the myocardium or be diffuse, and can occur at any agents can prevent the onset of myocarditis.
Patient Teaching
Emphasize the importance of rest and close monitor-
ing by the physician through follow-up appointments
during the recovery process. Give the patient a list of
the complications that should be reported to the phy-
sician, such as difficulty breathing, weakness, and
accumulation of fluid in the extremities.
Endocarditis
Description
Endocarditis is inflammation of the lining and the
valves of the heart (Figure 10-28).
ICD-9-CM Code 424.90
ICD-10-CM Code I38 (Endocarditis, valve
unspecified)
Endocarditis is coded by type and site. Refer to
the physician’s diagnosis and then to the current
manuals to ensure the greatest specificity. • Figure 10-28 Bacterial endocarditis. The valves are
covered with extensive vegetations. (From Damjanov I:
Symptoms and Signs Pathology for the health-related professions, ed 4, St Louis,
Endocarditis is usually secondary to infection else- 2011, Saunders/Elsevier.)
where in the body (Figure 10-29), the result of preex-
isting heart disease, or the consequence of an abnormal
immunologic reaction. The patient may have vague to
Respiratory tract infection Dental infection Urinary tract infection Skin infection
• Figure 10-29 Causative factors in endocarditis.
pronounced symptoms of infection, including fever, Stroke

chills, night sweats, weakness, anorexia, and fatigue. • Cerebral infarct
• Cerebral abscess Eye infarct
The condition is characterized by vegetative
Vegetations Vegetations
growths on the cardiac valves that may be released
into the bloodstream in the form of emboli. These
emboli can lodge in vessels and cause symptoms of
ischemia. The sites of ischemia may be the heart,
lungs, kidneys, brain, or extremities. Dysfunction of Aortic valve Mitral valve
the valves, which may not close effectively, disrupts or Myocardial
obstructs blood flow through the chambers of the Lung infarct infarct
Splenic
heart; this dysfunction of the valves usually produces Bacteremia infarct
a cardiac murmur heard on auscultation. Serious Renal
obstruction or regurgitation of blood flow through the infarct and
heart affects the pumping effectiveness of the heart Immunological lesions: pyelonephritis
• Glomerulonephritis
and causes complications. • Arthritis
• Dermal purpuric Osteomyelitis
Patient Screening vasculitis
A patient complaining of persistent fatigue, night
sweats, and/or fever requires prompt assessment by a
physician. Infarct of the
extremity
Etiology (gangrene)
Bacteremia, or the presence of infectious agents in the
bloodstream, can lead to endocarditis. Common Skin infarcts and
abscesses
infecting organisms include Staphylococcus aureus,
group A β-hemolytic streptococci, and Escherichia
coli. IV drug users are at high risk for fungal endocar-
ditis. Patients with damaged cardiac valves as a result
of rheumatic disease are more prone to endocarditis.
Septic emboli from endocarditis can be carried by the
arterial circulation and then embed in major organs, • Figure 10-30 Septic emboli from endocarditis are carried
by the arterial circulation and may lodge in major organs,
resulting in infarcts and new places of bacterial infec- causing infarcts and new sites of bacterial infection. (From
tions (Figure 10-30). Damjanov I: Pathology for the health-related professions,
ed 4, St Louis, 2011, Saunders/Elsevier.)
Diagnosis
A complete blood count (CBC) may indicate leuko- treat any complications. Bed rest is recommended
cytosis, and an elevated ESR may be present. Blood during the acute phase. Damaged cardiac valves may
cultures may reveal the causative organism. Echocar- need surgical repair or replacement.
diogram shows valve involvement with vegetation or
abscesses. ECG may indicate arrhythmia and conduc- Prognosis
tion defects. Early diagnosis and treatment with antibiotics usually
brings complete recovery. Untreated cases can have a
Treatment poor prognosis.
Identification of the causative organism dictates the
antiinfective therapy (usually IV antibiotics), which Prevention
continues for several weeks. Other medications include After recovery, the patient must understand the
antipyretics, anticoagulants, and drugs indicated to importance of taking prophylactic antibiotics before
dental work, childbirth, or any invasive procedures

associated with transient bacteremia. Patient Screening
New guidelines established in 2007 by the Patients with vague symptoms of fatigue, joint pain,
AHA no longer recommend antibiotics for many and fever after an episode of upper respiratory infec-
routine dental procedures. Prophylaxis is recom- tion and sore throat require prompt assessment by a
mended for dental procedures that involve manipu- physician.
lation of gingival tissue or the periapical region of
teeth or perforation of the oral mucosa. (This may Etiology
include routine cleanings, extractions, biopsies, suture After a sore throat caused by group A β-hemolytic
removal, and so on.) Always check the current guide- streptococcus, antibodies against the bacteria develop
lines for updates. and cross-react with normal tissue. This autoimmune
disease causes the antibodies to attack the body’s own
Patient Teaching cells and to initiate an inflammatory reaction. The
Instruct the patient to take the full course of antibiot- antibodies migrate to the endocardium and the mitral
ics and to obtain plenty of rest. and sometimes the aortic valves, where vegetations
form on the tissue. The carditis usually follows the
joint pain and fever by a week and can affect all layers
Rheumatic Fever of the heart (Figure 10-31).
Description Diagnosis
Rheumatic fever is a systemic inflammatory and auto- The history of an upper respiratory tract infection in
immune disease involving the joints and cardiac tissue. the preceding few weeks suggests rheumatic fever. No
single diagnostic feature identifies the condition. The
ICD-9-CM Code 390
presence of carditis and polyarthritis adds to the evi-
ICD-10-CM I00 (Rheumatic fever without
Code heart involvement) dence for the disease. The streptococcal antibody level,
Rheumatic fever is coded according to mention antistreptolysin O titer, is elevated in a series of tests.
of heart involvement. Refer to the physician’s Increases in cardiac enzyme levels, WBC count, and
diagnosis and then to the current editions of the ESR aid in the diagnosis.
ICD-9-CM and ICD-10-CM coding manuals to
ensure the greatest specificity of pathology. Treatment
After the diagnosis of streptococcal pharyngitis (strep
throat), which precedes rheumatic fever, treatment
Symptoms and Signs with a complete course of antibiotics prevents the
Rheumatic fever follows a sore throat caused by group onset of the fever and subsequent rheumatic heart
A β-hemolytic streptococcus. The patient, usually a disease. The administration of antibiotics (penicillin)
child, experiences a fever and polyarthritis, includ- is necessary to eradicate the streptococcal infection.
ing joint pain, edema, redness, and limited range Antipyretics are given for fever, and antiinflammatory
of motion. Joints frequently involved include finger, agents are given for relief of the arthritic symptoms.
knee, and ankle joints, with inflammation transient Bed rest is indicated, as is prophylactic administration
among these joints. In addition, the patient experi- of antibiotics.
ences carditis, cardiac murmurs, cardiomegaly, and
even CHF. Other symptoms include weakness, malaise, Prognosis
anorexia, weight loss, a rash on the trunk, abdominal The prognosis is good with treatment.
pain, and the development of small nodules on the
tendon sheaths in the knees, knuckles, and elbows. Prevention
The symptoms occur 1 to 5 weeks after the upper The prevention is prophylactic administration of anti-
respiratory tract infection. biotics for a diagnosed episode of strep throat.
medical attention for a child’s sore throats and to

include a β-hemolytic strep screen.
Rheumatic Heart Disease

Streptococcal
Group A
pharyngitis Description
Streptococcus
Rheumatic heart disease refers to cardiac manifesta-
Activation of tions that follow rheumatic fever.
T cells by
streptococcal ICD-9-CM Code 391
antigen ICD-10-CM Code I01.0 (Acute rheumatic
pericarditis)
Synthesis of (I01.0-I01.9 = 5 codes
antistreptococcal of specificity)
antibodies by
B cells Rheumatic heart disease is coded according to
Vegetation type and tissue involvement. Refer to the
Inflammation physician’s diagnosis and then to the current
Mitral leaflet
Short, thickened manuals to ensure the greatest specificity of
chordae tendineae
pathology.
Symptoms and Signs

The acute endocarditis, which leads to chronic cardiac
1 ENDOCARDITIS involvement, includes valvular damage, because the
Fibrinoid vegetations cause stenosis of the valves, particularly
material Giant the mitral and aortic valves (Figure 10-32). Rheumatic
Fibrosis
cell heart disease causes dyspnea, tachycardia, edema, a
Aschoff nonproductive cough, and cardiac murmurs.
bodies
Patient Screening
Lymphocyte Patients with vague symptoms of fatigue, joint pain, and
FIBRINOUS Macrophage fever after an episode of upper respiratory infection and
3 PERICARDITIS
2 MYOCARDITIS sore throat require prompt assessment by a physician.
• Figure 10-31 Pathogenesis of rheumatic fever. After
Etiology
infection (with “strep throat”), an immune response elicited
by the streptococci acts on the heart and several other After rheumatic fever, the vegetations may become
organs, most notably the joints, skin, and central nervous enlarged or the valves may scar, causing stenosis of the
system (CNS). In the heart, it causes endocarditis, myocar- openings. The frequency of rheumatic heart disease is
ditis, and pericarditis. (From Damjanov I: Pathology for the
health-related professions, ed 4, St Louis, 2011, Saunders/
decreasing as a result of diagnosis and aggressive anti-
Elsevier.) biotic treatment of streptococcal pharyngitis (strep
throat). Patients who experienced rheumatic fever and
rheumatic heart disease before the advent of penicillin
may have damaged cardiac valves (Figure 10-33).
Patient Teaching
Emphasize the importance of completing the full Diagnosis
course of the antibiotic therapy. Reassure the parents The diagnosis is based on the history of rheumatic
of the child with rheumatic fever that recovery usually fever and cardiac murmurs. An echocardiogram shows
follows medical treatment. Encourage parents to seek the vegetations or resulting damage to the valves.
Pulmonary valve
Aortic valve
Aortic Trucuspid
valve valve
Mitral
valve
Pulmonary
valve Mitral valve
Tricuspid
valve
Mitral valve
prolapse
• Figure 10-32 Cardiac valves.
Treatment
Treatment is aimed at reducing the stenosis of the
valves and preventing further damage. Surgery to
relieve the stenosis or to replace the valve may be
necessary. Good dental hygiene is important to prevent
gingival infection, which would cause further blood-
borne infection and damage the valves. Prophylactic
antibiotics are given to the patient before any dental
procedures.
Prognosis
The prognosis varies according to the extent of the
• Figure 10-33 Chronic rheumatic endocarditis of the damage to the valves. Recurrences are likely. Valve
aortic valve. The valves are deformed, and the orifice is replacement may give the patient a better outlook.
stenosed. (From Damjanov I, Linder J: Pathology: a color
atlas, St Louis, 1999, Mosby/Elsevier.)
Prevention
The prevention is prophylactic administration of anti-
biotics for a diagnosed episode of strep throat.
Patient Teaching NORMAL VALVE
Emphasize the importance of completing the full

course of antibiotic therapy. Encourage parents to seek
Blood flows freely forward No backflow of blood
medical attention for a child’s sore throats and to
include a β-hemolytic strep screen. Give information STENOSIS
on incision care to patients who have had valve
replacement surgery. Encourage the patient to comply
with prescribed rehabilitation therapy and activity Less blood flows through No backflow of blood
level. narrowed opening
INCOMPETENT VALVE
Valvular Heart Disease
Valvular heart disease is an acquired or congenital Blood flows freely forward Blood regurgitates
disorder that can involve any of the four valves of the backward through
heart (see Figure 10-32). This condition can occur in “leaky” valve
the form of either insufficiency or stenosis. Insuffi- EFFECT OF AORTIC STENOSIS
ciency, the failure of the valves to close completely,
allows blood to be forced back into the previous 4. Incomplete
chamber as the heart contracts. This exerts added pres- atrial
emptying
sure on that chamber and increases the heart’s work-
load. Stenosis, a hardening of the cusps of the valves
that prevents complete opening of the valves, impedes
the blood flow into the next chamber (Figure 10-34). 1. Narrowing of
The mitral valve is involved most often. The diagnosis 3. Decreased aortic valve
cardiac limits blood
of valvular heart disease requires ECG, radiographic output leaving the
chest studies, echocardiogram, and cardiac catheter- ventricle
ization. Treatment entails the administration of
digitalis or quinidine for arrhythmias and antibiotic
prophylaxis.
2. Left ventricular
hypertrophy
Mitral Stenosis
Description • Figure 10-34 Effects of heart valve defects. (From Gould
B: Pathophysiology for the health professions, ed 3, Phila-
Mitral stenosis is a hardening of the cusps of the mitral delphia, 2006, Saunders/Elsevier.)
valve that prevents a complete and normal opening
for the passage of blood from the left atrium into the
left ventricle. followed by hemoptysis. In severe cases, patients may
become cyanotic.
ICD-9-CM Code 394.0
ICD-10-CM Code I05.0 (Rheumatic mitral Patient Screening
stenosis)
Patients reporting exertional dyspnea and fatigue that
may be accompanied by palpitations require prompt
Symptoms and Signs assessment by a physician.
The mitral, or bicuspid, valve lies between the left
atrium and the left ventricle. Mitral stenosis causes Etiology
patients to have exertional dyspnea and fatigue. In Rheumatic heart disease is the cause of most cases of
addition, they may experience cough and palpitations mitral stenosis. The group A β-hemolytic streptococcus
stimulates antibody production, and the antibod- ICD-10-CM I05.1 (Rheumatic mitral
ies often attack the body tissue in an autoimmune Code insufficiency)
response. I05.2 (Rheumatic mitral stenosis
with insufficiency)
Diagnosis (I05.0-I05.9 = 5 codes of
specificity)
Symptoms of mitral stenosis may have an insidious or
an acute onset. A cardiac murmur is heard, including
a diastolic murmur. An echocardiogram confirms Symptoms and Signs
diagnostic suspicions. The patient with mitral insufficiency experiences
dyspnea and fatigue. A heart murmur can be heard as
Treatment the blood leaks back into the left atrium as a result of
Limitation of sodium intake, along with the adminis- the valve’s failure to close completely.
tration of diuretics, helps reduce the workload on the
heart. Anticoagulants prevent the formation of Patient Screening
thrombi. If atrial fibrillation results from stenosis, These individuals often call reporting that they
digoxin often is given to slow the rapid heart rate. are simply very tired and having some difficulty
Surgical intervention in the form of a commissurot- breathing. Anyone complaining of difficulty breathing
omy may be needed to free up the valve and to allow requires prompt assessment by a physician. Those
adequate blood flow; this can also be done nonsurgi- complaining of generalized fatigue should be sched-
cally with balloon valvuloplasty. Valve replacement is uled for the next available appointment.
a final option.
Etiology
Prognosis The valve may fail to close because of scar tissue result-
The prognosis improves with corrective intervention. ing from inflammation and vegetations (a consequence
of endocarditis), rheumatic fever, mitral valve prolapse
Prevention (MVP), CAD or MI, or from cardiac dilation.
Prevention of rheumatic fever and subsequent rheu-
matic heart disease is a major factor in preventing Diagnosis
mitral stenosis. The diagnosis is based on a thorough patient history,
especially a history of a sore throat or rheumatic fever.
Patient Teaching Physical examination reveals a murmur, and an echo-
When possible, use visual aids to demonstrate the cardiogram discloses the insufficiency. Cardiac status
effect valvular disease has on blood circulation through also is assessed with an ECG, radiographic chest film,
the heart. Explain the medical regimen and how medi- and cardiac catheterization.
cations can improve the condition. Explain the
procedure for surgical reconstruction of the valve if Treatment
appropriate. Treatment includes bed rest, oxygen therapy, and the
administration of antibiotics for any infectious process.
When severe, surgical repair or replacement of the
Mitral Insufficiency
valve may be necessary.
Description
In mitral valve insufficiency, the mitral valve fails to Prognosis
close completely and allows blood from the left ven- The prognosis generally is good, but the condition can
tricle to flow back into the left atrium. lead to CHF.
ICD-9-CM 394.1 (Rheumatic mitral
Code insufficiency)
Prevention
394.2 (Mitral stenosis with Preventing rheumatic fever and the resulting scarring
insufficiency) of the mitral valve help prevent mitral insufficiency.
Fused
posterior
medial
commissure
Pulmonary
artery
Left atrial
appendage
Fused anterior
lateral commissure
Anterior desc.
coronary artery
Mitral valve
Apex of left
ventricle
Dilator
E10-4 Mitral commissurotomy. (From Monahan: Phipps’ Medical-Surgical Nursing, ed 8, St. Louis, 2007,
Mosby.)
Patient Teaching Diagnosis

When possible, use visual aids to demonstrate the The typical click-murmur syndrome is heard on aus-
effect valvular disease has on blood circulation through cultation of the heart. An echocardiogram confirms
the heart. Explain the medical regimen and how medi- the failure of the valve to close. The premature
cations can improve the condition. Explain the pro- ventricular contractions (PVCs) that are detected on
cedure for surgical reconstruction of the valve if ECG are not considered harmful and are not an indi-
appropriate. cation of insult to the myocardium.
Treatment
Mitral Valve Prolapse
Treatment generally is not required for asymptomatic
Description patients. Those who experience discomfort and anxiety
MVP, usually a benign condition, occurs when one or often are treated with beta-blockers, and they are
more of the cusps of the mitral valve protrude back advised to avoid caffeine, smoking, and large, heavy
into the left atrium during ventricular contraction. meals.
ICD-9-CM Code 424.0 Prognosis
ICD-10-CM Code I34.0 (Nonrheumatic mitral
[valve] insufficiency) The prognosis is good.
I34.8 (Other nonrheumatic
mitral valve disorders)
Prevention
(I34.0-I34.9 = 5 codes of No prevention is known for this condition.
specificity)
Patient Teaching
Symptoms and Signs Reassure patients that this is usually a benign condi-
MVP, usually a benign condition, occurs when the tion. Emphasize the importance of avoiding caffeine
valve cusps do not close completely (see Figure 10-32). and big meals. The AHA no longer recommends
Most patients are asymptomatic, and the condition routine antibiotics before dental procedures or other
usually is discovered during a routine examination. surgical procedures for patients with only MVP, unless
The few patients who experience symptoms report they have had bacterial endocarditis in the past.
chest pain, dyspnea, dizziness, fatigue, and syncope.
These patients may experience severe anxiety. This
fairly common condition can affect all age groups. Arrhythmias
Patient Screening Description
Most individuals with MVP are unaware of any Cardiac arrhythmias are any deviation from the
problem. The condition is usually diagnosed inciden- normal heartbeat; that is, the normal sinus rhythm.
tal to a physical examination. Those who report chest They are often called irregular heartbeats.
pain require prompt assessment by a physician.
Etiology ICD-10-CM Code I47.1 (Supraventricular
tachycardia)
Abnormally long or short chordae tendineae may be
(I47.1-I47.9 = 3 codes of
the cause of the valve’s inability to close properly.
specificity)
Malfunctioning papillary muscles may increase the Arrhythmias or dysrhythmias are coded by type
severity of the condition. Regurgitation of blood and point of origin. Refer to the physician’s
occurs during left ventricular systole and results in the diagnosis and then to the current editions of the
rushing, gurgling cardiac murmur characteristic of the ICD-9-CM and ICD-10-CM coding manuals to
prolapse. ensure the greatest specificity.
diography may help confirm a particular arrhythmia

Symptoms and Signs (Figure 10-36). The patient may wear a Holter
Arrhythmias result when there is interference with the monitor (ambulatory ECG) to capture any arrhyth-
conduction system of the heart, resulting in an abnor- mic event.
mality of the heartbeat. Symptoms include palpita-
tions, rapid heartbeat (tachycardia), skipped heartbeats, Treatment
slow heart rate (bradycardia), syncope, and fatigue. Treatment depends on the cause (see Table 10-1).
Drug-induced arrhythmias usually resolve with ces-
Patient Screening sation of the drug administration. Anticoagulants,
Patients who report feeling abnormal heartbeats, especially warfarin (Coumadin), are given to prevent
usually as missed beats, palpitation, or rapid heart- a thromboembolism. Ischemia should respond to
beats, require prompt assessment by a physician. oxygen administration and increased blood flow to
the tissue. If the arrhythmia is unstable, cardioversion
Etiology may be performed by electric shock to the heart to
Arrhythmias can arise from disturbances in the normal restore normal heart rhythm. This mild shock inter-
conduction system of the heart, including the pace- rupts the arrhythmia pattern by resetting the heart
maker (the sinoatrial [SA] node), the atrioventricular rhythm and may enable the patient’s heart to be
(AV) node, the bundle branches, and the Purkinje normal enough to allow discontinuation of antiar-
fibers (Figure 10-35). Ischemia and drugs cause many rhythmic medications. Occasionally, the heart rhythm
arrhythmias. Failure of the SA node may be respon- does not stabilize, and the arrhythmia can be fatal.
sible. Table 10-1 lists the causes of arrhythmias. Heart Some arrhythmias can be treated with cardiac ablation
block occurs when the impulses from the SA node (radiofrequency ablation) techniques with catheters.
become slow or irregular at or below the AV node. Bradycardic heart rates may be treated by the inser-
tion of a pacemaker to maintain a rate at or above
Diagnosis 60 beats/minute. Implantable cardioverter defibrilla-
The diagnosis is made from a 12-lead ECG. Various tors may be used for those with tachycardic heart
arrhythmias are evident to the physician. Echocar- rates or ventricular arrhythmias. Refer to Table 10-1
for pacemaker application, implantable cardioverter-
defibrillator (ICD) application, and ablation proce-
Sinoatrial node dures. Refer to the Enrichment box about Pacemakers
and Implantable Cardioverter-Defibrillators and
Atrioventricular
node the Enrichment box about Cardiac Ablation
procedures for additional information. Common
Bundle of His
terms for ablation include cardiac catheter ablation,
radiofrequency ablation, cardiac ablation, or simply
ablation.
Left bundle
branch
Prognosis
Right bundle The prognosis varies depending on the type and cause
branch of the arrhythmia.
Prevention
Preventing drug-induced arrhythmias requires stop-
ping the intake of the offending drug substances.
Purkinje Avoiding the identified causative activities helps
fibers prevent certain arrhythmias. Certain types of arrhyth-
• Figure 10-35 Conduction system of the heart. mias may have no prevention.
TABLE
10-1 Arrhythmias
Symptoms
Types and Signs Etiology Diagnosis Treatment
Normal sinus Rate of 60-100 bpm, Impulse originates in Normal None indicated
rhythm regular, P wave SA node,
uniform conduction normal
Sinus tachycardia Rate >100 bpm, Rapid impulse Rapid rate Beta-blockers,
regular, P wave originates in SA CCBs; may be
uniform node, conduction candidate for ICD
normal
Sinus bradycardia Rate <60 bpm, Slow impulse Slow rate Atropine
regular, P wave originates in SA
uniform node, conduction
normal
Premature atrial Rate depends on Irritable atrium, single Irregular heartbeat, Treatment usually
contraction underlying rhythm, ectopic beat that diagnosis by unnecessary; if
usually normal P arises prematurely, ECG needed,
wave, different conduction antiarrhythmic
morphology from through ventricle drugs
other P waves normal
Atrial tachycardia Rate of 150- Irritable atrium, firing Rapid rate with Reflex vagal
250 bpm, rhythm at rapid rates, atrial and stimulation,
normal, sudden normal conduction ventricular rates calcium channel-
onset identical, blocking drugs
diagnosis by (verapamil),
ECG cardioversion; may
be candidate for
ICD or ablation
Atrial fibrillation Atrial rate >350 bpm, Atrial ectopic foci ECG shows no P IV verapamil; if
ventricular rate discharging at too waves, grossly unsuccessful,
<100 bpm rapid and chaotic irregular procainamide; if
(controlled) or rate for muscles to ventricular rate unsuccessful,
>100 bpm (rapid respond and cardioversion; may
ventricular contract, resulting be candidate for
response) in quivering of ICD or ablation
atrium; AV node
blocks some
impulses and
ventricle responds
irregularly
First-degree heart Rate depends on Delay at AV node, ECG shows P-R Atropine; if
block rate of underlying impulse eventually interval >0.20 unsuccessful,
rhythm, P-R conducted second artificial
interval >0.20 pacemaker
second insertion
TABLE
10-1 Arrhythmias—cont’d
Symptoms
Second-degree Intermittent block SA node initiates ECG shows Mild forms, no
heart block, with progressively impulse, normal P waves, treatment; severe,
Wenckebach longer delay in conduction some P waves insertion of
heart block conduction until through AV node not followed by artificial
one beat is is blocked QRS complex; pacemaker
blocked; atrial rate intermittently P-R interval
normal, ventricular progressively
rate slower than longer, followed
normal, rhythm by block of
irregular impulse
Classic second- Ventricular rate slow SA node initiates ECG shows P Artificial pacemaker
degree heart ( 12 , 12 , or 14 of impulse, waves present, is inserted
block atrial rate)— conduction QRS complex
rhythm, regular; P through AV node blocked every
waves normal, is blocked second, third, or
QRS complex fourth impulse
dropped every
second, third, or
fourth beat
Third-degree Atrial rate normal, SA node initiates ECG shows P Insertion of artificial
heart block ventricular rate impulse, which is waves and QRS pacemaker is
20-40 or 40- completely complexes with necessary
60 bpm; no blocked from no relationship
relationship conduction, to each other,
between P wave causing atria and rhythms are
and QRS complex ventricles to beat regular but
independently independent of
each other
Premature Single ectopic beat, Ectopic beat ECG shows a wide, Usually no treatment
ventricular arising from originates in bizarre QRS if <6/min and
contraction ventricle, followed irritable ventricle complex >0.12 single focus; may
(single focus) by compensatory second usually be candidate for
pause followed by a ICD or ablation
compensatory
pause
Multifocal Rate dependent on Same as single focus Same as single Same as single
arrhythmia underlying rhythm; (above) focus (above) focus (above);
Coupling, two rhythm regular or may be candidate
in a row irregular; P wave for ICD or ablation
Bigeminy, absent before
every other ectopic beat
beat
Trigeminy,
every third
beat
Quadrigeminy,
every fourth
beat
Continued
TABLE
10-1 Arrhythmias—cont’d
Symptoms
Ventricular Rate of Four or more ECG shows runs Often forerunner
tachycardia 150-250 bpm, consecutive PVCs of four or more of ventricular
rhythm usually at a rapid rate due PVCs, P wave fibrillation;
regular; focus of to advanced buried in QRS immediate
pacemaker irritability of complex intervention
normally single, myocardium, necessary—IV
patient indicating lidocaine; if
experiences ventricular unsuccessful,
palpitations, command of heart follow by
dyspnea, and rate cardioversion;
anxiety followed procainamide or
by chest pain bretylium may be
used; this is a
candidate for ICD
or ablation
Ventricular Patient loses Ventricular fibers Pulseless, Recognize and
fibrillation (a consciousness twitch rather than unconscious terminate rhythm;
lethal immediately after contract, reason patient; ECG precordial shock
arrhythmia) onset; no unknown shows rapid, (defibrillation);
peripheral pulses repetitive, survivors become
palpable, no heart chaotic waves candidates for ICD
sounds, no blood originating in or ablation
pressure ventricle
AV, Atrioventricular; bpm, beats per minute; CCB, calcium channel blocker; ECG, electrocardiogram; ICD, implantable cardioverter-
defibrillator; IV, intravenous; PVCs, premature ventricular contractions; SA, sinoatrial.
Patient Teaching ICD-10-CM R57.9 (Shock, unspecified)

Code R57.0 (Cardiogenic shock)
Give patients information about the causes of arrhyth- R57.1 (Hypovolemic shock)
mias. Emphasize the importance of complying with R57.8 (Other shock)
prescribed drug therapy. Encourage patients to comply Refer to the physician’s diagnosis and then to
with scheduled appointments. Emphasize the symp- the current editions of the ICD-9-CM and
toms that require prompt medical intervention. ICD-10-CM coding manuals to ensure the
Shock
Description Symptoms and Signs
Shock is the collapse of the cardiovascular system, Shock causes inadequate perfusion of organs and
including vasodilation and fluid shift, accompanied by tissues. The patient has pale, cold, and clammy skin;
inefficient cardiac output. a rapid, weak, and thready pulse; rapid breathing; and
ICD-9-CM 785.50 (Shock unspecified, an altered level of consciousness. The blood pressure
Code failure of peripheral drops, and the patient may be anxious, irritable, or
circulation) restless and often expresses a feeling of impending
785.51 (Cardiogenic shock) doom. The patient may experience dizziness, extreme
785.59 (Other) thirst, and profuse sweating. In late stages, the pupils
P T
Atrial Ventricular
depolarization Q S repolarization
and contraction Ventricular and relaxation
depolarization
and contraction
SA node
Right Atrial muscle
atrium
AV node
Internodal Bundle of His
tracts Left bundle branch
Purkinje fibers ECG tracing
(ventricular
muscle)
Right bundle branch
Right ventricle
• Figure 10-36 Conduction system in the heart and relationship to electrocardiogram (ECG). AV, Atrio-
ventricular; SA, sinoatrial. (From Gould B: Pathophysiology for the health professions, ed 3, Philadelphia,
2006, Saunders/Elsevier.)
ENRICHMENT
Pacemakers and Implantable Cardioverter-Defibrillators
Implantable electronic cardiac assist devices have progressing to ventricular fibrillation and even to
extended the lives of those with cardiac arrhythmias. cardiac arrest.
At the present time, there are two programmable The artificial pacemaker helps to relieve symptoms
devices that can be surgically implanted for regulation of heart rhythm bradycardic disturbances. As normal
of heart rates and arrhythmias. In an emergent heart rates and rhythms are restored, circulation of
situation, a temporary pacemaker may be used until a blood returns to normal and symptoms of dizziness,
permanent pacemaker or implantable cardioverter- shortness of breath, and fatigue typical of bradycardia
defibrillator (ICD) can be implanted. usually are abated. The pacemaker also provides
Assisting the heart’s natural pacemaker, the treatment for sick sinus syndrome.
sinoatrial (SA) node, and when necessary the rest of ICDs are used to treat tachyarrhythmias, rapid
the impulse conduction system, artificial pacemakers heartbeats that usually result from heart disease
can be external (a temporary intervention) or including coronary artery disease (CAD), myocardial
implanted. These devices are used to treat infarction (MI), or cardiomyopathy. The rapid rates
arrhythmias, heart block, and sick sinus syndrome. originating in the ventricle result in the chambers of
ICDs are implanted when ventricular tachycardia has the heart not filling adequately, resulting in less blood
the potential to become irregular and unstable, reaching the brain, pounding heart, and faintness or
Continued
ENRICHMENT—cont’d
dizziness. When this rhythm becomes irregular and This information is reviewed by the physician and any
unstable, the ventricle beats in a disorganized changes in the settings of the devices may be made.
manner, resulting in no blood being pumped out of Regular pacemaker checks are conducted according
the heart. This is very rapidly followed by cardiac to an established schedule. A programming head is
arrest. The ICD contains an internal defibrillator that placed over the pacemaker. ECG leads remain on
will automatically pace or shock the heart out of the the arms and chest allowing the clinician to view
dangerous or lethal rhythm. An additional feature of information collected and stored in the recording
the ICD is that it also is responsive to slow heart rates device in the pacemaker. The computers in the office
and at the interpretation of bradycardia, the ICD will make a recording of the activity since the last check.
act as a pacemaker. Current pacemaker settings are viewed and reset if
Pacemakers and ICDs are small electronic required. The condition of the battery is checked,
battery powered units that are implanted usually just including predicted end of service date or estimated
under the skin of either side of the chest wall. The battery life. Precautions are necessary for patients
lead that transmits impulses from the pacemaker or with implanted pacemakers and ICDs.
ICD to the wall of the heart is threaded through the Electromagnetic fields are to be avoided, because
subclavian vein, the superior vena cava, into the right they can interfere with the settings of the units. At the
atrium of the heart where it is attached to the wall of present time, magnetic resonance imaging (MRI)
the right atrium. When a block of impulses exists as scans should not be performed on patients with
in heart block, a dual-chamber pacemaker with two implantable cardioverter defibrillator devices. However,
lead wires will be implanted. The second lead is new technology has emerged with MRI-compatible
threaded along with the first into the right atrium, then pacemakers and lead systems. Individuals with the
into the right ventricle where it is attached to the wall devices should avoid magnetic or electronic security
of that chamber. The single-chamber pacemaker scanning and identify themselves (using a pacemaker
sends impulses to initiate the heartbeat in the atrium. identification card) as individuals with implantable
When there is a block, either at or below the pacemakers and request a “pat down” or hand-held
atrioventricular (AV) node, the second lead will screening. Cellular phones may present a problem
transmit impulses to stimulate the contraction of the when held within 6 inches of the unit. Therefore,
right ventricle. Most pacemakers have a rate- individuals with implantable cardiac assist devices
responsive feature to adjust the rate of pacing should always use the phone with the ear on the
according to the activity of the individual’s body. opposite side of the implant site. Other precautions
Sensors in the unit monitor changes in the body and include not arc welding with the cables draped over
adjust the rate according to the body’s need for the neck, not working with a demagnetizer, and not
adequate perfusion. The leads for the ICD run through using a gas powered chainsaw. All of these activities
the right chambers of the heart and end in the apex may create an electromagnetic field that can disturb
of the right ventricle. the settings of the unit.
Pacemakers and ICDs contain small It is important that the patient and the family
microprocessors, batteries, and electronic circuitry. receive instructions on pacemaker or ICD restrictions.
Both store the history of the heart’s and the device’s These devices have allowed many to live normal lives
activities, which are retrieved at the pacemaker clinic. with their cardiac arrhythmias.
ALERT!
Caution during Procedures Utilizing of the electrical activity to the pacemaker. The
Electrical Activity defibrillator interprets the electrical activity as
Individuals with pacemakers and/or implantable ventricular fibrillation and shocks the individuals.
defibrillators make all health care providers that they Any procedure involving electrical activity should be
have the implantable devices. Any procedure utilizing performed in an outpatient setting at a hospital type
electrical activity may cause the devices to fail. setting where a special magnet can be placed over
Pacemakers interpret the electrical activity as heart the device. The magnet prevents the electrical activity
beats and will stop pacing. The impact depends on from reaching the device.
the duration of the electrical activity and the proximity
E10-5 Protecta™ XT DR and VR ICDs with SmartShock

Technology™ are designed to reduce dramatically the
number of inappropriate shocks received by patients being
treated for tachycardia. (Courtesy David Frazier, 2012.)
ENRICHMENT
Cardiac Ablation
Patients with abnormal heart rhythms may be impulses. Should it be necessary during the
candidates for cardiac ablation. Drug therapy is procedure, the electrodes can pace the heart
usually attempted to correct the arrhythmia. When (cause it to beat). This study, using several electrode
there is little or no response, cardiac ablation may be catheters, helps to isolate where the aberrant
attempted. pathway is located.
Cardiac ablation is a nonsurgical procedure that The ablating catheter is introduced into the heart
uses catheters inserted into the chambers of the close to where the abnormal electrical pathway is
heart to administer energy to destroy the small located. Radiofrequency energy is passed through the
amount of tissue that causes electrical rhythm catheter, causing the tip of the catheter to heat up.
disturbances in the cardiac conduction system. The area of the heart containing the abnormal
The procedure is performed by an pathway is destroyed. A scar that cannot conduct the
electrophysiologist after he or she completes electrical impulse results and that pathway can no
“mapping” of the heart’s electrical system. The longer cause the arrhythmia.
electrophysiologist watches the procedure with the Ablation is used to treat tachycardias, both atrial
aid of fluoroscopy, inserting an electrode catheter and ventricular. The idea is to obliterate the focus that
usually through a vein (sometimes an artery) and is firing too rapidly and prevent the impulse from
threading it into the heart chambers. The electrode spreading through the heart. Other common terms
catheters sense the electrical activity in various are cardiac catheter ablation, radiofrequency ablation,
areas of the heart and measure the speed of the cardiac ablation, or simply ablation.
dilate, the eyes become dull and lusterless, and the supply to the circulating system can generate shock
patient experiences shaking and trembling. (Figure 10-37).
Patient Screening Diagnosis

Shock is a life-threatening condition requiring the The clinical picture, along with a history of a precipi-
patient’s immediate entry into the emergency medical tating event, leads to the diagnosis of shock resulting
system. Assist the caller in contacting emergency from inadequate cellular perfusion. The altered level
medical service (EMS). of consciousness and respiratory distress suggests
shock. Immediate intervention is needed to halt the
Etiology progression of the condition.
This life-threatening emergency can be caused by ana-
phylaxis, hemorrhage, sepsis, respiratory distress, Treatment
heart failure, neurologic failure, emotional catastro- Because of the severity and rapid progression of the
phe, or severe metabolic insult. Regardless of the condition, aggressive intervention is undertaken at the
cause, the amount of blood that is effectively circulat- earliest possible opportunity. The CABs (compres-
ing in the body is reduced. The final effect is that sions, airway, and breathing) of emergency care require
the vital organs (heart, brain, lungs, and kidneys) do maintaining an open airway and establishing ventila-
not receive sufficient oxygen and nutrients to sustain tion to supply the vital organs with oxygen. Any visible
life. Rapid blood loss or significant fluid loss with bleeding is controlled, and surgical intervention may
subsequent hypovolemia precipitates shock. Failure be needed to halt internal bleeding. The patient should
of the heart to pump adequately is another cause be placed in a supine position, with the feet and legs
of shock. Vascular collapse with subsequent massive elevated, and should be kept warm but not overheated.
dilation or constriction of the vessels can cause blood If the patient is not in an inpatient facility, contacting
to pool away from vital areas. Insufficient oxygen the EMS for immediate transport to an emergency
DECREASED BLOOD PRESSURE
STIMULATE SYMPATHETIC NERVOUS SYSTEM

Thirst
Anxiety, restlessness
Tachycardia
Vasoconstriction, pallor
COMPENSATIONS RENIN–ANGIOTENSIN–ALDOSTERONE
to maintain Vasoconstriction
heart and
Retention of Na+ and water, oliguria
brain functions
INCREASED ADH SECRETION

Retention of water
DIRECT EFFECTS OF DECREASED BLOOD PRESSURE

Lethargy, weakness
Anaerobic metabolism Metabolic
acidosis
VASODILATION AND DECREASED CELL FUNCTION

Slow blood flow in microcirculation Thrombus forms
Ischemia in organs Decreased function
Necrosis (e.g., kidney)
DECREASED VENOUS RETURN
FURTHER DECREASE IN CARDIAC OUTPUT

Severe acidosis
CNS depression
Organ damage (e.g., acute renal failure, lung damage)
DECOMPENSATION
• Figure 10-37 Progress of shock. ADH, Antidiuretic hormone; CNS, central nervous system. (From
Gould B: Pathophysiology for the health professions, ed 3, Philadelphia, 2006, Saunders/Elsevier.)
facility is indicated. Vital signs are monitored, and failure, certain arrhythmias, or valve failure can lead
volume replacement is instituted with IV fluids. When to cardiogenic shock (Figure 10-38).
supplemental oxygen is available, it is administered.
Diagnosis
Prognosis The clinical picture and a history of a major cardiac
Immediate assessment and intervention improves the insult lead to the suspicion of cardiogenic shock. An
prognosis for complete or near-complete recovery. ECG is another diagnostic tool, as are radiographic
When the condition is not addressed promptly, shock chest studies. A hypotensive state that continues to
may become unstoppable and the consequences may worsen also indicates the diagnosis.
be irreversible, resulting in an unfavorable outcome.
Treatment
Prevention Treatment consists of general measures for shock,
Most occurrences of shock are not preventable. Imme- along with the administration of medications that
diate intervention helps prevent rapid progression of increase the efficiency of the myocardium and/or
the condition into a fatal occurrence. affect the blood vessels. Blood supply to vital organs
must be improved, and the oxygen demands of myo-
Patient Teaching cardial tissue must be reduced. Blood volume assess-
Give patients and family members information about ment determines whether drugs are given to dilate or
causes and emergency intervention measures for constrict cardiac vessels. Sometimes an intraaortic
patients with known allergic reactions. Encourage balloon pump may be inserted.
community education in first aid and CPR to help
reduce damaging outcomes of shock. Prognosis
The prognosis varies but in many cases is
unfavorable.
Cardiogenic Shock
Description Prevention
Cardiogenic shock is the inadequate output of blood Prevention in most cases is unlikely. An important
by the heart. factor in preventing a fatal outcome is immediate
assessment and intervention when symptoms and
ICD-9-CM Code 785.51
signs are identified.
ICD-10-CM Code R57.0 (Cardiogenic shock)
Patient Teaching
Symptoms and Signs Emphasize the importance of immediate emergency
In cardiogenic shock (shock resulting from inadequate intervention when symptoms are detected. Promote
cardiac output), the myocardium fails to pump effec- community awareness of the benefits of first aid
tively. The patient exhibits the previously mentioned and CPR training in increasing survival rates in the
symptoms and signs of shock. The event usually is community.
preceded by MI or severe heart failure, certain arrhyth-
mias, or acute valve failure. Cardiac Tamponade
Patient Screening Description
These patients have a life-threatening condition. Enter Cardiac tamponade is the compression of the heart
them promptly into the emergency medical system. muscle and restriction of heart movement caused by
blood or fluid trapped in the pericardial sac. It may
Etiology be called cardiac compression.
Any insult that disturbs the heart’s ability to pump ICD-9-CM Code 423.3
blood can cause cardiogenic shock. MI, severe heart ICD-10-CM Code I31.4 (Cardiac tamponade)
Normal circulation
Hypovolemic shock
To brain
To brain
Decreased
venous
return
R L Decreased blood
R L
volume
Capillaries
Capillaries
Decreased blood flow
Neurogenic or vascular shock
Cardiogenic shock To brain
To brain
Decreased
pumping force General
vasodilation
leads to
increased
R L capacity
of system
L Heart damage
R Capillaries
Capillaries
• Figure 10-38 Causes of shock. (From Gould B: Pathophysiology for the health professions, ed 3,
Philadelphia, 2006, Saunders/Elsevier.)
Symptoms and Signs Prevention

Cardiac tamponade occurs when a coronary, epicar- Because of the etiology of this condition, prevention
dial, or pericardial vessel breaks and blood is trapped usually is not possible.
in the pericardial sac. In addition, the myocardium
may rupture, also sending blood into the pericardial Patient Teaching
sac. The blood constricts the heart, thus restricting Give the patient and family information about the
heart movement, and less blood can enter the heart mechanism and results of the insult. Explain care of
chambers per beat. The patient with cardiac tampon- the incision and emphasize the importance of follow-
ade experiences sudden severe dyspnea and rapidly ing postoperative orders.
falling blood pressure. The pulse becomes weak, If available, provide the patient and family with
thready, and rapid. The patient is in shock and some form of computer-based health education about
becomes cyanotic above the nipple line. The level of postoperative care.
consciousness falls.
Vascular Conditions
Patient Screening
These patients have a life-threatening condition. The vascular system, a closed transport system com-
Enter them promptly into the emergency medical posed of arteries, arterioles, capillaries, venules, and
system. veins, is responsible for supplying tissues with blood
containing oxygen and nutrients. This system also
Etiology conveys waste products and carbon dioxide to the
Cardiac tamponade may result from an insult to the appropriate organs for excretion. Arteries carry blood
integrity of a vessel in the pericardium, allowing blood away from the heart (Figure 10-39, A), veins transport
to fill the pericardial space. The pressure of the blood blood back to the heart (see Figure 10-39, B), and
causes the heart to beat inappropriately, leading to capillaries are the point of exchange at the cellular level
cardiac arrest. Cardiac tamponade may also occur in (see Figure 10-39, C).
patients with certain cancers, chronic kidney failure, Blood vessel walls, other than one-cell-walled
and other medical conditions, such as hypothyroidism capillaries, are composed of three layers: the tunica
or lupus. intima, the tunica media, and the tunica externa
(Figure 10-40). The lining of the vessel lumen, the
Diagnosis tunica intima, is composed of smooth, thin endothe-
The diagnosis is based on the clinical picture and a lium, allowing minimal friction with the flowing
history of a traumatic event. Heart sounds become blood. The middle portion, the tunica media, is com-
muffled or distant, but breath sounds remain normal. posed of smooth muscle and elastic tissue that is under
the control of the sympathetic nervous system. This
Treatment innervation allows constriction or dilation of the
Treatment consists of inserting a needle into the vessel walls and changes in blood pressure. Connective
pericardial space and withdrawing the offending tissue composes the outermost layer, the tunica
blood. Surgery usually is indicated to repair the externa, creating support and protection for the
leak. vessels.
Arterial walls are much thicker than venous walls.
Prognosis The tunica media is heavier in arteries to compensate
The prognosis varies and depends on the causative for the stronger blood pressure under which the arter-
factor, the success of the intervention, and the health ies must function. Veins, with their lower blood pres-
status of the patient. Surgical repair usually has a posi- sure, contain valves to prevent backflow and to assist
tive outcome. the return of blood to the heart (Figure 10-41).
E10-6 Cardiac tamponade. (From Mosby’s Dictionary of

Medicine, Nursing and Health Professionals, ed 9, St. Louis,
2009, Mosby.)
A B
C Venule Capillary Arteriole

• Figure 10-39 Vascular system. A, Arteries. B, Veins. C, Capillary exchange.
Endothelium ICD-9-CM Code 444 (Requires fourth digit)

ICD-10-CM Code I74.0 (Embolism and
thrombosis of
Valve
abdominal aorta)
Tunica
intima (I74.0-I75.89 = 20 codes
of specificity)
Tunica Emboli are coded according to location of
intima Elastic
layer involvement in the arterial system. Refer to the
physician’s diagnosis for the location of arterial
Tunica Tunica involvement and then to the current editions of
media media
the ICD-9-CM and ICD-10-CM coding manuals
to ensure the greatest specificity.
Tunica Tunica
externa externa Symptoms and Signs
Symptoms of emboli depend on the location of the
occluded vessel and the magnitude of the area of tissue
Vein Artery
served by the vessel. The initial symptom is severe pain
in the area of the embolus. Emboli lodging in arter-
ies of the extremities cause the area to become pale,
numb, and cold to the touch. In addition, arterial
Capillary
pulses are absent below the occlusion if it is arte-
rial. When a large artery is involved, the patient also
• Figure 10-40 Vessel wall structure. experiences nausea, vomiting, fainting, and eventu-
ally shock. Pulmonary obstructions are discussed in
Chapter 9. Cerebral obstructions and CVAs are dis-
cussed in Chapter 13.
Patient Screening
A patient reporting severe pain in an extremity that is
accompanied by paleness, numbness, and coolness in
the area requires prompt assessment by a physician. In
addition, those experiencing nausea, vomiting, faint-
ing, and shock are in an emergency state and require
immediate entrance into the emergency medical
Open Closed system.
• Figure 10-41 Venous valves.
Etiology
Vascular conditions include emboli, arteriosclero- Emboli are usually blood clots, but the offending
sis, atherosclerosis, aneurysms, phlebitis, thrombo- embolus also may be composed of air bubbles, fat
phlebitis, varicose veins, thromboangiitis obliterans globules, bacterial clumps, or pieces of tissue, includ-
(Buerger disease), and Raynaud disease. ing placenta. The most common offender is a venous
thrombosis, a blood clot that has formed in the deep
veins of the legs as a result of venous stasis (Figures
Emboli
10-42, 10-43, and 10-44). A portion of the thrombus
Description breaks loose from the clot and travels through the
Emboli are clots of aggregated material (usually venous system until it becomes lodged in a vessel that
blood). They can lodge in a blood vessel and inhibit is too narrow to allow passage, often in the lungs.
the blood flow. Cardiac arrhythmias also can cause thrombi to form
Carotid and
E cerebral
arteries
Coronary arteries D
Aortic
atherosclerosis
C
Aortic
aneurysm
Valvular thrombi
(vegetations)
Thrombus over
myocardial infarct
A Varicose veins
• Figure 10-42 Common sites of thrombus formation. (From Damjanov I: Pathology for the health-related
professions, ed 4, St Louis, 2011, Saunders/Elsevier.)
in the heart. Those that travel from the left ventricle in a patient who has any of the aforementioned pre-
can enter the coronary arteries, resulting in an MI, or dispositions is another clue.
can enter the carotid and cerebral arteries, compro-
mising blood supply and resulting in CVAs. Treatment
Treatment depends on the area of involvement. Treat-
Diagnosis ment of pulmonary embolism (see Chapter 9), MI,
The clinical picture and a history of bed rest, physical and CVA (see Chapter 13) is aggressive and immediate
inactivity, heart failure, arrhythmias, and any condi- if the patient is to survive. The treatment of a patient
tion that has put pressure on or decreased flow in the with an arterial embolus in an extremity is also aggres-
veins of the legs or pelvis alert the physician to the sive and immediate to prevent the death of tissue and
possibility of an embolus. Pain in the calf of the leg eventual gangrene. Blood flow is reestablished to the
Venous embolism Arterial embolism
SYMPTOMS OF
PULMONARY
EMBOLISM Brain infarct
• Shortness of
breath
• Hemoptysis
• Pain Lung
• Sudden death infarct
Saddle
embolus
Ventricular
thrombus
Splenic infarct
Kidney infarct
Intestinal infarct
Venous
thrombus
Infarct of the
extremity
• Figure 10-43 Venous and arterial emboli. (From Damjanov I: Pathology for the health-related profes-
sions, ed 4, St Louis, 2011, Saunders/Elsevier.)
affected part by lowering the limb, wrapping it to main- reteplase, or streptokinase), can be administered via a
tain warmth to the area, and treating any constriction central catheter directly into the spot of the coronary
of blood vessels. Heparin or enoxaparin (Lovenox) embolism to break down the emboli.
are administered to deter further clot formation, and
antispasmodic drugs are given for vascular spasms. If Prognosis
this therapy is not successful, surgical intervention The prognosis varies and depends on the location of
may be indicated to remove the obstruction and to the emboli. When the embolus is in an extremity,
restore circulation. In another option, a thrombolytic aggressive treatment and/or surgical intervention
drug (such as urokinase, TPA, tenecteplase, alteplase, usually is successful.
Thrombus
Atherosclerosis
lodges in lung Description
Atherosclerosis, a thickening and hardening of the
arteries, occurs when plaques of cholesterol and lipids
form in the arterial tunica intima.
Embolism travels
Clot forms in from legs or pelvic ICD-9-CM Code 440 (Requires fourth digit)
pelvic veins veins up inferior ICD-10-CM Code I70.0 (Atherosclerosis of
vena cava through
right side of heart aorta)
to lungs Atherosclerosis is coded according to the site.
Symptoms and Signs

Atherosclerosis is responsible for most myocardial and
cerebral infarctions. The patient with atherosclerosis
• Figure 10-44 Venous thrombosis. often is asymptomatic. The first symptoms may be
angina pectoris, dizziness, elevated blood pressure,
and shortness of breath.
Prevention Patient Screening

Preventing the formation of deep vein thrombosis Patients reporting symptoms of angina pectoris, diz-
during periods of immobilization or reduced physical ziness, elevated blood pressure, and shortness of breath
activity is essential. require prompt assessment by a physician. Those who
are asymptomatic may report just not feeling well;
Patient Teaching after careful questioning, these patients should be
Encourage those who will be traveling and sitting for scheduled as soon as possible or referred to a facility
long periods of time to get up and walk every hour where they can be seen quickly.
for a few minutes. If this is not possible, suggest exer-
cises that stimulate contraction of the calf muscles. Etiology
The etiology of atherosclerosis is multifactorial and
complicated, but there are risk factors that appear
Arteriosclerosis
to increase the risk that the condition will develop.
Description Heredity seems to increase the occurrence, as do a
Arteriosclerosis is a group of diseases characterized by sedentary lifestyle, a diet rich in lipids and cholesterol-
hardening of the arteries. Arteriosclerosis has three producing foods, cigarette smoking, diabetes mellitus,
forms: atherosclerosis, Mönckeberg’s arteriosclerosis, hypertension, and obesity. The lipids and cholesterol
and arteriolosclerosis. Atherosclerosis occurs when in the blood form thick, stiff, and hardened lesions in
plaques of fatty deposits form within the arterial the medium-size and large arteries. The lesions are
tunica intima. Mönckeberg’s arteriosclerosis, medial eccentric and eventually expand to occlude the artery
calcific sclerosis, involves the arterial tunica media; (Figure 10-45). A fatty streak forms in the arterial wall
there is destruction of muscle and elastic fibers along and migrates to the tunica intima. Plaque forms and
with calcium deposits. Arteriolosclerosis occurs when thickens the arterial wall. A sequela is an ulceration,
the walls of the arterioles thicken, resulting in loss of crack, or fissure in the plaque where platelets can
elasticity and contractility. aggregate and form a thrombosis. Ischemia results
Normal arterial Atherosclerotic Advanced arterial

lumen plaque deposit atherosclerotic
disease
• Figure 10-45 Atherosclerosis.
from reduced blood supply to the dependent tissue, dietary changes. It is possible with good compliance
with resulting pain. Infarct occurs with advanced to prevent progression of the disease.
occlusion of the artery and is followed by tissue
necrosis. Prevention
Prevention is important, as is education about risk
Diagnosis factors and changes in lifestyle.
The diagnosis often is made during a routine physical
examination or screening process. Blood studies indi- Patient Teaching
cate elevated cholesterol, triglyceride, and lipid levels. Emphasize the importance of following dietary and
Hypertension may be noted. Doppler studies of major exercise recommendations. If the patient is a diabetic,
vessels show reduced blood flow. explain the need to keep blood glucose levels in
normal range. Inform smokers of the dangers that
Treatment smoking poses to their health. Give details about
Treatment consists of dietary changes to reduce satu- agencies that offer patients additional information
rated fats and food high in cholesterol and lipids. about the condition and support groups in the
Cigarette smokers are encouraged to stop smoking. community.
Hypertension and diabetes mellitus are treated and
controlled. Hyperlipidemic drugs such as lovastatin,
Aneurysms
simvastatin, pravastatin, rosuvastatin, or atorvastatin
are prescribed. Recent additions to the recommended Description
drug therapy include a niacin and lovastatin combina- An aneurysm is a weakening and resulting local dila-
tion (Advicor) and ezetimibe (Zetia), a drug that tion of the wall of an artery (Figure 10-46).
inhibits the absorption of cholesterol in the intestine.
Other therapies include cholestyramine, colesevelam, ICD-9-CM Code 442.9 (Of unspecified site)
colestipol, or omega-3 ethyl esters. Research is being ICD-10-CM Code I72.9 (Aneurysm of
unspecified site)
conducted to confirm claims that hyperlipidemic drug
(I72.0-I72.9 = 7 codes of
therapy can bring about regression of the condition
specificity)
and actually reverse the plaque buildup. Aneurysms are coded according to site and
type. Refer to the physician’s diagnosis for site
Prognosis and type of aneurysm and then to the current
The prognosis varies and depends on patient compli- editions of the ICD-9-CM and ICD-10-CM coding
ance with prescribed drug therapy, exercise, and manuals to ensure the greatest specificity.
Aortic arch Patient Screening

Ascending aorta Patients reporting a pulsating mass in the abdomen
require prompt physician assessment. Those with
symptoms of impending or evolving rupture require
immediate entry into the emergency medical system.
Etiology
Saccular
aneurysm A common cause of aneurysms is a buildup of athero-
sclerotic plaque that weakens the vessel wall. Trauma,
Fusiform infection or inflammation, and congenital tendencies
aneurysm
are additional causative factors.
Diagnosis
The aortic mass is noted midabdomen, and pulsation
is observed. A bruit heard on auscultation is another
sign of the arterial dilation. Cerebral aneurysms
Dissecting
aneurysm usually are discovered when they rupture with cata-
strophic consequences. Radiographic studies, ultraso-
nography, CT, and magnetic resonance imaging
(MRI) all help to confirm the diagnosis. The ruptured
False aneurysm precipitates symptoms and signs of shock.
aneurysm
Treatment
Treatment depends on the size, location, and likeli-
hood of rupture of the defect. Most diagnosed aortic
• Figure 10-46 Types of aortic aneurysms.
aneurysms should be treated with surgical repair
before they leak or rupture. A newer form of treatment
with catheter-based stent grafts is now available for
Symptoms and Signs many patients as an alternative to surgery. After the
The symptoms of an aneurysm may have either an integrity of the aortic wall has been breached, immedi-
insidious or a sudden, acute onset. Symptoms depend ate surgical intervention is required to repair the
on the location and size of the aneurysm and the rupture—usually with a synthetic graft—if the patient
extent of the dilation. Abdominal aortic aneurysm is is to survive. Watchful waiting often is employed
the most common form. An asymptomatic aneurysm when the aneurysm itself is small or is in a small vessel.
of the aorta often is discovered during a routine physi-
cal examination when the abdomen is being palpated Prognosis
or as the result of an abdominal radiographic study The prognosis varies depending on the location and
conducted for another reason. As the aortic aneurysm extent of the aneurysm. Surgical intervention before
enlarges, the patient may experience abdominal or rupture or severe leakage of the vessel creates a better
back pain, and a pulsating mass is observed in the prognosis than when the aneurysm has ruptured or is
abdomen. A complication of any aneurysm is leakage leaking large amounts of blood. The speed of emer-
from the wall of the artery or sudden rupture of the gency intervention also affects the prognosis.
weak area. When this occurs, the patient exhibits
symptoms and signs of hemorrhagic shock. Rupture Prevention
of a cerebral aneurysm mimics signs of a CVA, with Prevention is unlikely as the condition is the result of
unilateral neurologic deficits being noted. many uncontrollable factors.
not to massage the affected tender area because this

Patient Teaching manipulation may stimulate the formation of clots or
Instruct those who have had surgery about postopera- the release of formed clots as emboli.
tive care of the incision. Encourage compliance with
any prescribed drug therapy, dietary modifications, Prognosis
and exercise regimens. The prognosis is usually positive with treatment.
Prevention
Phlebitis
Because the cause is uncertain, prevention is probably
Description not possible.
Phlebitis, an inflammation of a vein, occurs most
often in the lower legs, but any vein, including cranial Patient Teaching
veins, may be affected. Give patients and family members information
warning against massaging the affected area.
ICD-10-CM Code I80.9 (Phlebitis and
thrombophlebitis of Thrombophlebitis
unspecified site)
Phlebitis is coded by site. Refer to the physician’s
Description
diagnosis for location of phlebitis and then to the Thrombophlebitis is the result of inflammation of
current editions of the ICD-9-CM and ICD-10-CM a vein with the formation of a thrombus on the
coding manuals to ensure the greatest specificity. vessel wall.
Symptoms and Signs ICD-9-CM Code 451 (Requires fourth

digit)
Superficial vein involvement results in pain and ten- ICD-10-CM Code I80.00 (Phlebitis and
derness in the affected area and becomes more severe thrombophlebitis of
as the condition progresses. Swelling, redness, and superficial vessels of
warmth are noted, followed by the development of a unspecified lower
tender cordlike mass under the skin. extremity)
Deep venous inflammation affects the tunica intima, (I80.00-I80.9 = 31
allowing the formation of clots (thrombophlebitis). codes of specificity)
Thrombophlebitis is coded by site. Refer to the
Patient Screening physician’s diagnosis for location of phlebitis and
then to the current editions of the ICD-9-CM and
Patients reporting swelling, redness, warmth, and pain
in a limb require prompt assessment by a physician.
greatest specificity of pathology.
Etiology
The cause is uncertain, and the condition may appear Symptoms and Signs
for no apparent reason. Possible causes include venous Thrombophlebitis causes an interference with blood
stasis, obesity, blood disorders, injury, and surgery. flow and resulting edema. As with phlebitis, the
patient experiences pain, swelling, heaviness, and
Diagnosis warmth in the affected area along with chills and fever.
The clinical picture and history of a preceding event The involved area is tender to palpation.
help establish the diagnosis.
Patient Screening
Treatment Patients reporting swelling, redness, warmth, and
Treatment of superficial phlebitis is symptomatic, pain in a limb require prompt assessment by a
with analgesics given for pain. Caution must be taken physician.
Etiology Patient Teaching

Venous stasis, blood disorders that cause a hyperco- Encourage those who will be traveling and sitting for
agulable state, and injury to the venous wall play long periods of time to get up and walk every hour
important roles in the occurrence of thrombophlebi- for a few minutes. If this is not possible, suggest exer-
tis. The deep venous inflammation of phlebitis affects cises that stimulate contraction of the calf muscles.
the tunica intima, allowing the formation of clots Emphasize the importance of immediate attention
(thrombophlebitis). should the patient experience a recurrence of the
symptoms.
Diagnosis
The clinical picture of gross edema in one leg, result-
Varicose Veins
ing in a measurable difference in the circumference of
the legs, suggests thrombophlebitis. The affected area Description
is tender to palpation. Imaging of the vessel with Varicose veins are swollen, tortuous, and knotted veins
radiographic venography and ultrasonography con- that usually occur in the lower legs (Figure 10-47).
firms the diagnosis.
Treatment ICD-10-CM I83.009 (Varicose veins of
Code unspecified lower extremity
Immediate intervention is necessary. The affected part with ulcer of unspecified
is immobilized to prevent the thrombus from spread- site)
ing and dislodging to become an embolus. Heparin is I83.019 (Varicose veins of
administered to prevent the clot from enlarging, and right lower extremity with
antibiotics are given to prevent infection. ulcer of unspecified site)
Prognosis
Prompt intervention usually leads to a positive
outcome. The condition usually resolves, and no
further treatment is indicated. If the condition does
not resolve, surgical intervention may be needed to
ligate the affected vessel. Collateral circulation devel-
ops. As with emboli, preventing the formation of deep
vein thrombosis during periods of immobilization or
reduced physical activity is essential.
Prevention
Prevention of thrombophlebitis includes implement-
ing activities that encourage movement of legs during
long periods of sitting such as on an airplane or on a
long auto trip. If unable to walk around during this
time, exercise the leg muscles regularly to encourage
blood to flow back to the heart and to prevent a blood
clot from forming. Individuals who will be sitting for
extended periods of time are encouraged to force Normal Distended
valve valve
fluids preventing dehydration.
Females using oral contraceptives are encouraged
not to smoke while taking the medication as it
increases the chances of blood clots that may become Normal leg veins Varicose veins of leg
thrombi from forming. • Figure 10-47 Varicose veins.
E10-7 Varicose veins. (From Forbes: Color Atlas and Text of

Clinical Medicine, ed 2, London, 1998, Mosby.)
I83.029 (Varicose veins of left prolonged standing, prolonged sitting, or pregnancy

lower extremity with ulcer are usually all that is needed to make the diagnosis.
of unspecified site) Advanced varicosities cause the skin around the
(I83.001-I83.93 = 57 codes affected areas to take on a brown discoloration.
of specificity)
Varicose veins are coded by type. Refer to the Treatment
physician’s diagnosis for specific site and then to
the current editions of the ICD-9-CM and Rest periods throughout the day, with the patient
ICD-10-CM coding manuals to ensure the lying down and elevating the feet higher than the
greatest specificity. heart, afford relief in mild cases. Engaging in exercise
and submerging the legs in warm water increases the
flow of blood. The patient may wear support stockings
Symptoms and Signs that encourage the return flow of blood. Those who
Symptoms of varicose veins develop gradually, with have to stand for long periods are instructed to move
a feeling of fatigue in the legs, followed by a continu- the legs at frequent intervals to stimulate the muscular
ous dull ache. Leg cramps may be experienced at “milking” of the veins.
night, and the ankles may swell. As the condition Painful, twisted, and swollen veins that have pro-
progresses, the veins thicken and feel hard to the gressed beyond the treatment of rest and exercise
touch. Pain worsens and can have a dull or stabbing usually require surgical intervention in the form of a
quality. vein ligation and stripping or injection of sclerosing
solutions that harden and eventually atrophy the
Patient Screening affected veins. Collateral circulation develops to
These patients usually are not in acute distress and can augment the blood return to the heart.
be scheduled for the next available appointment.
Should they report swelling accompanied by pain and Prognosis
redness in the area, prompt assessment by a physician Treatment, including venous ligation or injection of
is suggested. sclerosing solutions, usually affords relief for the
symptoms and resolution to the condition.
Etiology
Varicose veins have no clearly identifiable cause. Prevention
However, defective or absent valves may be suspected. Wearing support stockings and moving the legs
Prolonged standing or sitting causes pressure on during long periods of standing help prevent this con-
the valves in the superficial veins of the lower legs. dition. Some individuals, however, may be predis-
Normal movement of the legs causes the muscles to posed to the condition, especially during pregnancy.
contract and relax, thus “milking” the blood upward. Weight loss that relieves abdominal pressure also
When the person stands or sits for extended periods, helps.
gravity pushes the blood downward, with resulting
pressure on the valves. Without the normal muscular Patient Teaching
contractions, the venous walls distend, reaching a Give patients information about preventing stasis of
point at which they and the valves are no longer blood flow in the legs. Give information on care of
competent. Stasis of blood follows, causing the swell- postoperative incisions.
ing of the veins. The enlarging uterus during preg-
nancy increases the pressure on the leg veins and pelvic Thromboangiitis Obliterans
veins, compromising the free flow of the venous
(Buerger Disease)
blood.
Description
Diagnosis Thromboangiitis obliterans (Buerger disease) is an
The presence of the twisted, swollen, knotted veins of inflammation of the peripheral arteries and veins of
the lower legs on clinical inspection and a history of the extremities with clot formation (Figure 10-48).
Diagnosis
The diagnosis follows reports of intense pain, usually
in the legs or the instep. An arteriogram and other
studies, such as an ultrasonogram, identify the site of
the clot or obliteration. The ulcers on the skin are
another indication of the disease and its severity. The
history of long-term smoking of tobacco products
suggests the diagnosis.
Treatment
The first step in treatment is the immediate and complete
cessation of smoking. This often reduces the inflamma-
tion and restores partial circulation to the area. Several
medications, including prasugrel or clopidogrel, may
• Figure 10-48 Thromboangiitis obliterans (Buerger
disease). The lumen is occluded by a thrombus containing
improve circulation. Buerger-Allen exercises also help to
two abscesses. The vessel wall is infiltrated by leukocytes. improve circulation to the area. In these exercises, the
(From Kumar V, Cotran R, Robbins S: Robbins basic pathol- patient elevates the feet and legs 45 degrees to 90 degrees
ogy, ed 8, Philadelphia, 2008, Saunders/Elsevier.) until they blanch and then lowers them below the rest
of the body until they redden. The patient then rests in
ICD-9-CM Code 443.1
a supine position. If these steps do not restore circulation,
ICD-10-CM Code I73.1 (Thromboangiitis
Obliterans [Buerger’s
surgical intervention to establish detours or to bore a
Disease]) pathway through the clot itself may be necessary. Ampu-
tation of gangrenous tissue is imperative.
Symptoms and Signs
The patient experiences intense pain in the affected Prognosis
area, usually the legs or the instep of the foot, which No cure is known for this condition, but cessation of
is aggravated by exercise and relieved by rest. If the smoking relieves symptoms. Surgical intervention
condition is not resolved and circulation is not restored may help. When treatment fails, amputation may be
to the affected area, atrophy, ulcers, and even gangrene necessary.
can develop.
Prevention
Patient Screening Because the primary cause is smoking of tobacco
Patients, especially males, reporting intense pain in products, prevention requires not smoking or cessa-
the limbs that is aggravated by exercise and relieved tion of smoking.
by rest usually are scheduled for the next available
appointment. Those complaining of breakdown of Patient Teaching
skin tissue require prompt assessment by a physician. Give information about the care of foot injuries and
instructions about avoiding constrictions around the
Etiology affected limb.
The primary cause of Buerger disease is long-term
smoking of tobacco. The inflammation and resulting
Raynaud Disease
clot formation in the vessels continue to advance until
the entire vessel is obliterated and circulation to the Description
area is completely compromised. The ischemic tissue Raynaud disease is a vasospastic condition of the
dies, and gangrene follows. This condition affects pri- fingers, hands, or feet. It causes pain, numbness, and
marily males, most often those of Jewish descent. sometimes discoloration in these areas.
A B
E10-8 Buerger’s disease. (From Goldman: Goldman’s Cecil Medicine, ed 24, Philadelphia, 2012,
Saunders.)
ICD-9-CM Code 443.0 to stop smoking, to avoid exposure to cold, and to

ICD-10-CM I73.00 (Raynaud’s syndrome avoid stressful situations that bring on the attacks.
Code without gangrene) Drug therapy to dilate vessels and to increase blood
(I73.00-I73.01 = 2 codes of flow includes vasodilators, α-adrenergic blockers, and
specificity)
CCBs. The side effects of the drug therapy may limit
their use to treat the condition.
Symptoms and Signs
This bilateral condition is precipitated by cold and Prognosis
causes a blanching (white) discoloration, followed by The painful event resolves with application of warmth
blue as venous blood remains, and finally red or purple to the affected area. The condition will continue to
when circulation is restored. The attacks occasionally recur, however, with exposure to cold and as long as
are triggered by stressful events. Raynaud disease is the individual continues to smoke.
much more common in women than in men. When
the disorder is primary, it is called Raynaud disease; Prevention
when it is secondary to another disease, it is called Preventing the attacks requires avoiding exposure to
Raynaud phenomenon. In severe cases, the digits may cold and wearing gloves and heavy socks and shoes for
ulcerate and become very painful. In most cases, the protection from the extreme cold. Cessation of smoking
prognosis is good. also helps prevent recurrences of the condition.
Patient Screening Patient Teaching

Those who have been diagnosed usually know they Give patients information about the effect of smoking
need to warm the area gently. Those experiencing on their blood vessels. Encourage them to avoid direct
the condition for the first time and experiencing skin exposure to extreme cold and to wear gloves,
the severe pain of the condition require prompt heavy socks, and warm shoes to protect the fingers,
intervention. hands, feet, and toes.
Etiology
Blood Dyscrasias
The small peripheral arteries and arterioles supplying
the fingers, hands, and feet spasm and constrict, com- Blood is composed of formed elements, RBCs (eryth-
promising the circulation to these appendages. The rocytes), WBCs (leukocytes), platelets (thrombo-
spasm follows exposure to cold or possibly results from cytes), and a liquid portion (plasma). It is responsible
a stressful occurrence. The episode usually resolves for transporting vital elements, including oxygen,
spontaneously after the application of warmth. The nutrients, and hormones, to the body cells. It also
condition also is made worse by smoking tobacco. plays a part in the removal of waste products, in the
inflammatory response, and in the function of the
Diagnosis immune system. In addition, blood helps to maintain
The diagnosis is based on the clinical picture and a homeostasis, acid-base and fluid balance, and body
history of numbness and paleness of the areas. Normal temperature.
arterial pulses are present. The condition most often Blood is synthesized by the hematopoietic system
affects women between puberty and the age of 40 in the bone marrow (myeloid) and lymphoid tissue
years, especially those who smoke. In severe cases, the found in the lymph nodes, spleen, thymus, bone
compromise in circulation can lead to tissue necrosis marrow, and gastrointestinal (GI) tract (Figure 10-49).
and even amputation. The reticuloendothelial system is found in the spleen,
liver, lymph nodes, and bone marrow. It is responsible
Treatment for removing worn-out blood cells from the blood-
Treatment of the episode involves the application of stream and breaking down the blood cell components
warmth to the affected areas. Patients are encouraged for recycling or elimination from the body.
E10-9 Raynaud’s phenomenon in the acute phase, with

severe blanching of the tip of one finger. (From Forbes: Color
Atlas and Text of Clinical Medicine, ed 2, London, 1998,
Mosby.)
Child
Adult
• Figure 10-49 Sites of blood cell formation.
Stem cells in bone marrow form blast cells in- factors. Normal values of blood components are mea-
cluding erythroblasts (rubriblasts), which eventually sured by specific laboratory testing.
become erythrocytes, and myeloblasts, which eventu-
ally become leukocytes (Figure 10-50).
Anemias
Deviation or malfunctioning in this system results
in various blood dyscrasias, either by impairment in Description
the formation of the blood components or by unusual Anemia is defined as a condition in which there is a
destruction of the cells. Dyscrasias involving eryth- reduction in the quantity of either RBCs or hemoglo-
rocytes and platelets are anemias, thrombocytoses, bin in a measured volume of blood, reducing the
thrombocytopenias, and polycythemias. Leukocyte blood’s ability to carry oxygen to the cells.
dyscrasias include leukemias, lymphomas, and leu- Depending on the severity of the anemia, one or
kopenia. Bleeding and clotting problems arise from many symptoms may occur, including pallor, fatigue,
alterations affecting thrombocytes and plasma-clotting dizziness, shortness of breath, and irregular heartbeats.
Hemocytoblast
Myeloid Lymphoid
Stem Cells Stem Cells
Progenitor Progenitor Progenitor
Proerythroblast Megakaryoblast Myeloblast Monoblast Lymphoblast
Erythroblast
stages
Basophilic Eosinophilic Neutrophilic
Megakaryocyte myelocyte myelocyte myelocyte
Promonocyte Prolymphocyte
Ejection
of nucleus
Basophilic Eosinophilic Neutrophilic
Reticulocyte
band cell band cell band cell
Erythrocytes Platelets Basophil Eosinophil Neutrophil Monocyte Lymphocyte
Granulocytes Agranulocytes
Red Blood
Cells (RBCs) White Blood Cells (WBCs)
• Figure 10-50 Formation of blood cells. (From Patton KT, Thibodeau GA: Anatomy and physiology,
ed 9, St Louis, 2016, Mosby.)
The treatment varies with the cause. Several possible Anemias are coded by type. Refer to the
causes are acute or chronic blood loss, impaired pro- physician’s diagnosis for specific type of anemia
duction of RBCs (including aplastic anemia, iron- and then to the current editions of the ICD-9-CM
deficiency anemia, anemias of chronic disease, or and ICD-10-CM coding manuals to ensure the
greatest specificity of pathology.
megaloblastic anemia), inherited or acquired hemo-
lytic conditions, anorexia nervosa, and hemolytic-
hemoglobin disorders. Hemoglobin in RBCs is Symptoms and Signs
necessary to transport oxygen to all cells, and any Regardless of the cause, anemic patients experience
condition that reduces the amount of hemoglobin fatigue. Most appear pale. As the disease progresses,
results in anemia. Iron and other components are the symptoms become more pronounced, and the
needed to synthesize the hemoglobin. patient may have dyspnea, tachycardia, and a pound-
Types of anemias include iron deficiency, folic acid ing of the heart. Pallor is noted on the palmar surface
deficiency, pernicious, aplastic, sickle cell, hemor- of the hands and in the nail beds, conjunctiva, and
rhagic, and hemolytic. mucous membranes of the mouth.
Important presenting symptoms that tend to recur
in patients with anemia and need further investigation Patient Screening
include: Anemias often are diagnosed incidental to a physical
• Fatigue examination or during evaluation of other disorders.
• Dyspnea (difficulty in breathing) The individual reporting dyspnea, tachycardia, and a
• Headache “pounding” heart requires prompt assessment by a
• Loss of appetite physician. Those reporting they are pale and “just
• Heartburn plain tired” should be scheduled at the next available
• Edema, especially of the ankles appointment.
• Numbness and tingling sensations
• Syncope (fainting) Etiology
• Pallor Anemias are classified by the color of the RBC
as hypochromic, normochromic, or hyperchromic
ICD-9-CM Code 280-289 (spherocytosis), and by size as microcytic, normocytic,
ICD-10-CM Code D50.0 (Iron deficiency or macrocytic (Figure 10-51). They also are classified
anemia secondary to
by the causative factor, as previously mentioned.
blood loss [chronic])
Iron-deficiency anemia may be secondary to blood
D51.0 (Vitamin B12
deficiency anemia due loss through hemorrhage, a slow insidious bleed such
to intrinsic factor as bleeding hemorrhoids, and even heavy menstrual
deficiency) flow, or because of insufficient intake of dietary iron.
D58.0 (Hereditary Folic acid deficiency anemia results when insuffi-
spherocytosis) cient amounts of folic acid are available for DNA
D59.0-D59.1 synthesis, thus preventing the maturation of the blood
(Autoimmune hemolytic cells. This can be the consequence of a dietary defi-
anemia) ciency and is clinically similar to pernicious anemia.
D64.0-D64.3 Pernicious anemia is considered a macrocytic
(Sideroblastic anemia)
anemia, in which immature RBCs are larger than
D66 (Hereditary factor VIII
normal. The hemoglobin volume is reduced, with sub-
deficiency)
D69.0 (Allergic purpura) sequent reduction in oxygen-carrying capacity. This
D70.9 (Neutropenia, anemia is considered the result of an autoimmune
unspecified) response and is discussed in Chapter 3.
D75.1 (Secondary Autoimmune hemolytic anemia also is considered an
polycythemia) autoimmune response and is discussed in Chapter 3.
Normal erythrocytes
Normocyte
Microcyte
Sickled erythrocytes
• Figure 10-52 Sickled erythrocytes.
hemolytic anemias, but exposure to chemical toxins

Macrocyte
and certain bacterial toxins or autoimmunity also may
be the cause.
• Figure 10-51 Sizes of erythrocytes. Diagnosis
Blood studies show reduced RBC numbers, reduced
Aplastic anemia results from an insult to the hema- hemoglobin levels and hematocrit, and changes in
topoietic cells (stem cells) in the bone marrow. Eryth- the morphology of the corpuscles (Table 10-2).
rocyte, leukocyte, and thrombocyte production is Bone marrow studies may be ordered to detect any
reduced because of exposure to myelotoxins, such as aberrations.
benzene, alkylating agents, antimetabolites, certain
drugs and insecticides, and radiation. Treatment
Sickle cell anemia, a chronic hereditary hemolytic Treatment is directed at the cause of the anemia.
form of anemia, is found predominately in those of Dietary or supplemental iron administration is benefi-
the black race. The presence of hemoglobin S along cial in iron-deficiency anemias. If anemia is the result
with hemoglobin A is noted in erythrocytes, causing of a slow insidious bleed, the underlying cause must
them to acquire a sickle or elongated shape on deoxy- be found and treated. Folic acid replacement is indi-
genation (Figure 10-52). These rigid misshapen cells cated in folic acid deficiency anemia. Vitamin B12
obstruct capillary flow and lead to tissue hypoxia and injections are the treatment of choice for pernicious
further sickling; this in turn causes further obstruction anemia. The causative factor must be uncovered in
and eventually infarction. When the sickling causes an hemolytic and aplastic anemias and, if possible, elimi-
obstruction, the individual experiences severe pain. nated. No cure is known for sickle cell anemia, so
When the sickled cells are reoxygenated, they resume treatment is symptomatic. Rest, increased fluid intake,
the natural round disk shape of a normal erythrocyte. and the administration of analgesics are helpful. When
In addition, hemoglobin S has reduced oxygen- the condition is exacerbated, the patient requires hos-
carrying capacity. pitalization for oxygen administration, IV therapy, the
Hemorrhagic anemia results from a large decline use of narcotic analgesics, and packed RBC transfu-
in blood volume (hypovolemia) in a short time. sion. In all forms of anemia, blood replacement may
Hemolytic anemia is caused by abnormal destruc- be needed when other measures fail to restore the
tion of the RBCs. Heredity plays a role in some RBC numbers and hemoglobin levels.
E10-10 Sickle cell. (From Rodak BF, Carr JH: Clinical

Hematology Atlas, ed 4, St. Louis, 2013, Saunders.)
TABLE
10-2 Blood Values in Anemia
Mean
Mean Mean Corpuscular
Corpuscular Corpuscular Hemoglobin White Reticulocyte Platelet
Red Blood Hemoglobin Hematocrit Volume Hemoglobin Concentration Blood Cell Count Count
Cell (per mm3) (g/dl) (%) (per mm3) (pg) (g/dl) (per mm3) (per mm3) (per mm3)
Normal Male: 4.7-6.1; Male: 14-18; Male: 42-52; 80-90 27-31 32-36 5000- 0.5-2 150,000-
female: female: female: 10,000 400,000
4.2-5.4 12-16 37-47
Acute Initial increase, Initial Normal Increase Decrease Normal Increase Increase Decrease
hemorrhagic latent decrease, initially,
anemia decrease latent latent
decrease decrease
Chronic Decrease Decrease Decrease Slight Slight Slight decrease Normal Decrease Normal
hemorrhagic decrease decrease
anemia
Iron-deficiency Decrease Decrease Decrease Decrease Decrease Decrease Normal Decrease Normal to
anemia increase
Aplastic Gross decrease Gross Gross Moderate Gross Gross decrease Gross Decrease Gross
anemia decrease decrease decrease decrease decrease decrease
Pernicious Decrease Gross Gross Increase Increase Increase Slight Decrease Slight
anemia decrease decrease decrease decrease
Folic acid Decrease Gross Gross Increase Increase Increase Slight Decrease Slight
deficiency decrease decrease decrease decrease
anemia
Sickle cell Decrease Decrease Decrease Decrease Normal Normal Increase Increase Normal
anemia
Hemolytic Decrease Decrease Decrease Increase Slight Normal Normal Increase Normal to
anemia decrease increase
with chemotoxic agents can be a cause. Benzene is

Prognosis another chemical agent that causes neutropenia.
The prognosis varies and depends on the cause and Agranulocytosis, or neutropenia, is an acute insult to
type of anemia and the patient’s response to prescribed the body’s immune system and drastically reduces the
therapy. body’s response to bacterial infection. The infectious
agents usually invade the body through the oral or
Prevention pharyngeal mucosa and often are already present but
Prevention also depends on the type and cause of the are no longer held in check by the now-absent granu-
anemia. Most are not preventable. Proper nutrition locytes. Patients occasionally are sensitive to a particu-
may play a role in preventing anemia caused by low lar drug, such as chlorpromazine, propylthiouracil,
iron intake. phenytoin, chloramphenicol, and phenylbutazone.
Agranulocytosis also can accompany aplastic or mega-
Patient Teaching loblastic anemia, tuberculosis, uremia, or malaria.
Give patients information about proper nutrition. This condition occurs most often in the female
Emphasize the importance of complying with pre- population.
scribed drug therapy.
Diagnosis
Blood studies show leukopenia, with a substantial
Agranulocytosis
decrease in the number of polymorphonuclear cells.
Description Bone marrow studies reveal a lack of granulocytes, and
Agranulocytosis (also called neutropenia) is a blood the developing WBCs are not mature and are reduced
dyscrasia in which leukocyte levels become extremely in number. History may reveal exposure to the offend-
low. ing infectious agents; blood, urine, and oral cultures
may be positive for bacteria; and toxins may be
ICD-9-CM Code 288.0 (Requires fifth digit)
present.
ICD-10-CM Code D70.9 (Neutropenia,
unspecified)
Treatment
(D70.0-D70.9 = 7 codes
of specificity) The primary thrust of treatment is to eliminate any
offending microorganism through aggressive antimi-
crobial therapy. Cultures are repeated several times
Symptoms and Signs and are monitored for the growth of microbes. If the
This condition can have a rapid onset. The patient cause of the toxicity is identified as a drug or chemical,
experiences severe fatigue and weakness, followed by exposure to the toxic agent must be halted. Aggressive
a sore throat, ulcerations on the oral mucosa, dyspha- therapy is indicated because the condition can be fatal
gia, elevated body temperature, weak and rapid pulse, within a week if left untreated.
and chills.
Prognosis
Patient Screening The prognosis varies and depends on the etiology of
Patients reporting severe fatigue and weakness fol- the condition. Cessation of exposure to a toxic sub-
lowed by a sore throat, ulcerations on the oral mucosa, stance often produces improvement. As previously
dysphagia, an elevated body temperature, weak and mentioned, some cases of this condition may be fatal
rapid pulse, and chills require prompt assessment by within a week if left untreated.
a physician.
Prevention
Etiology Prevention involves avoiding the toxic substance
Agranulocytosis usually is caused by drug toxicity or after it has been identified. Prevention is not always
hypersensitivity. For example, cancer chemotherapy possible.
Patient Teaching Diagnosis

Give the patient information about exposure to the An abnormal increase in RBC numbers, hemoglobin
toxic substance. Help the patient and family find levels, and hematocrit suggests the condition. Leuko-
information about exposure to hazardous materials. cyte and thrombocyte counts also are elevated, and the
spleen may be enlarged. The clinical picture aids in
the diagnosis. The total RBC mass evaluation is also
Polycythemia
diagnostic.
Description
Polycythemia (polycythemia vera) is an abnormal Treatment
increase in the amount of hemoglobin, the RBC Periodic phlebotomy is employed to reduce the blood
count, or the hematocrit, causing an absolute increase volume. Myelosuppressive drugs and radiation also
in RBC mass. improve the blood count. Relative polycythemia usually
subsides when the causative factors are eliminated.
ICD-9-CM Code 238.4
ICD-10-CM Code D45 (Polycythemia vera) Prognosis
The periodic phlebotomies help to reduce the RBC
Symptoms and Signs count and hematocrit. This treatment is a required
Symptoms of polycythemia are related to the increased lifelong protocol for many patients. Some cases will
RBC mass and include headaches, dyspnea, irritabil- resolve, as previously mentioned.
ity, mental sluggishness, dizziness, syncope, night
sweats, and weight loss. Circulatory stagnation, Prevention
thrombus, and increased blood viscosity may be With no known etiology, no prevention is known.
noted. Splenomegaly and clubbing of the fingers along
with cyanosis may be observed. Patient Teaching
Emphasize the importance of routine blood cell
Patient Screening counts and the need for continued phlebotomy.
Patients reporting headaches, dyspnea, irritability, Encourage patients to keep routine appointments.
mental sluggishness, dizziness, syncope, night sweats,
and weight loss require prompt assessment. Schedule Thrombocytopenia
an appointment as soon as possible. If a prompt
appointment is not possible, refer the patient to a Idiopathic thrombocytopenic purpura is considered
facility where assessment can be performed quickly. an autoimmune response. This dyscrasia involving
reduced clotting capabilities of the blood is discussed
Etiology in Chapter 3.
It is not known why a sustained increase in the hema-
topoiesis of the bone marrow causes absolute, or Leukemias
primary, polycythemia. Relative polycythemia results
when the plasma volume is reduced by dehydra- Leukemias are malignant neoplasms of the blood-
tion, plasma loss, fluid and electrolyte imbalances, or forming organs (bone marrow, spleen, and lymph
burns. Reduced oxygen supply to the tissues results in nodes) that produce an abnormal, uncontrolled,
a compensation by the body as it manufactures addi- clonal proliferation of one specific type of blood cell
tional hemoglobin to carry additional oxygen. The in the lymphoid or myeloid cell lines. The large
body uses this compensatory process when patients number of leukemic cells leads to bone marrow over-
have chronic pulmonary and cardiac diseases or live crowding, resulting in reduced production and func-
at high altitudes, where the oxygen concentration is tion of normal blood cells. The reduced numbers of
reduced. functional neutrophils, erythrocytes, and platelets
result in frequent infections, anemia, and clotting mediastinal mass can be found in many patients with
problems. the T cell ALL.
Leukemias are classified by the cell type and the
degree of differentiation of the neoplastic cells. Acute Patient Screening
leukemias are composed of immature-appearing, Patients complaining of bone pain, weight loss,
large hematopoietic cells (blasts). Chronic leukemias fatigue, night sweats, weakness, persistent fevers, a
produce more mature-appearing, yet hypofunctional, tendency toward increased bruising and bleeding,
cells. Acute disease has a rapid progression and can be recurrent infections, headache, blurred vision, and/or
quickly fatal (with a natural history of 1 to 5 months), cranial nerve palsies require prompt assessment by a
whereas chronic leukemias have a slower progression physician.
measured in years rather than months. Common types
of leukemia include acute lymphocytic leukemia, Etiology
chronic lymphocytic leukemia, acute myelogenous ALL accounts for 20% of adult leukemias and is the
leukemia, and chronic myelogenous leukemia. most common childhood leukemia. Childhood ALL
is covered in more detail in Chapter 2. The exact cause
is unknown. Prolonged exposure to radiation, certain
Acute Lymphocytic Leukemia
chemicals and drugs, smoking, viruses, and genetic
Description factors (certain chromosomal abnormalities) are con-
Acute lymphocytic leukemia (ALL) is characterized by sidered contributing factors.
an overproduction of immature lymphoid cells (lym-
phoblasts) in the bone marrow and lymph nodes. It Diagnosis
is generally classified into two subtypes depending on A peripheral blood smear shows increased numbers of
the type of cell affected: B cell ALL or T cell ALL. immature lymphocytes and reduced numbers of
erythrocytes and platelets. Microscopic examination
ICD-9-CM 204.0 (Acute lymphoid leukemia)
of a bone marrow aspiration or biopsy is necessary for
Code (Requires fifth digit)
ICD-10-CM C91.00 (Acute lymphoblastic definitive diagnosis. Blast cells should make up more
Code leukemia not having achieved than 25% of all nucleated cells in the bone marrow
remission) to diagnose ALL. Cerebrospinal fluid (CSF) should be
(C91.00-C91.02 = 3 codes of withdrawn and examined for leukemic cells. A karyo-
specificity) type is performed to look for any abnormalities that
Refer to the physician’s diagnosis for specific may help determine prognosis and treatment options.
type of lymphoid leukemia and then to the
current editions of the ICD-9-CM and ICD- Treatment
10-CM coding manuals to ensure the greatest Treatment regimens for ALL involve aggressive che-
specificity of pathology.
motherapy for 2 to 3 years consisting of three phases:
induction, consolidation, and maintenance. Because
Symptoms and Signs CNS invasion is common in ALL, the CNS is treated
The patient appears pale and may report bone pain, with intrathecally administered chemotherapy even if
weight loss, sore throat, fatigue, fever, night sweats, blasts are not noted in the CSF. Those with the Phila-
and weakness. There is a tendency toward increased delphia chromosome are often treated with tyrosine
bruising and bleeding. As the leukemic cells infiltrate inhibitors. Hematopoietic stem cell transplantation
the spleen, liver, lymph nodes, and nervous system, (HSCT) is an option for patients who have relapsed
they interfere with the normal functioning of these or for adults with poor prognostic features.
organs. Lymphadenopathy and splenomegaly are
common at presentation. Symptoms of central nervous Prognosis
system (CNS) involvement include headache, blurred The overall 5-year survival rate is 78% to 85%, with
vision, nausea, vomiting, and cranial nerve palsies. A children generally having a better prognosis than
adults. Adults often have cytogenetic abnormalities

that carry a worse prognosis than the ones found in Patient Screening
children. In addition, children often can tolerate rela- Patients complaining of weight loss, fever, night
tively higher doses of the drugs currently used to treat sweats, extreme fatigue, and noticeable swelling of
ALL than adults can. Poor prognostic indicators cervical, supraclavicular, or axillary lymph nodes
include age of less than 1 year, CNS invasion, presence require prompt assessment by a physician.
of the Philadelphia chromosome, or other adverse
abnormalities, and failure to achieve remission after Etiology
induction therapy is completed. CLL is the most common leukemia in adults. The
median age at diagnosis is 70 years, and there is
Prevention a male predominance. Having a first degree rela-
No prevention methods are known for ALL. tive with CLL increases the risk of a person devel-
oping CLL.
Patient Teaching
Give patients and parents of children information Diagnosis
about the chemotherapy. Help them find and contact Nearly 95% of cases are discovered incidentally during
community support groups. routine blood work. Peripheral blood smear with or
without bone marrow studies is performed and reveal
an increased number of mature-appearing lympho-
Chronic Lymphocytic Leukemia
cytes. In most cases of CLL, the absolute lymphocyte
Description count is greater than 5,000/μl and the bone marrow
Chronic lymphocytic leukemia (CLL) is a neoplasm is hypercellular with lymphocytes accounting for more
that involves the lymphocytes. It is a slowly progress- than 30% of all nucleated cells. Because CLL most
ing disease that results in the accumulation of mature- often involves the B-lymphocytes, screening for the
appearing, but hypofunctional, lymphocytes. presence of certain B-cell markers is performed. A
karyotype and testing for certain genetic mutations are
ICD-9-CM 204.1 (Chronic lymphoid
obtained.
Code leukemia) (Requires fifth digit)
ICD-10-CM C91.10 (Chronic lymphocytic Many staging systems for CLL have been pro-
Code leukemia of B-cell type not posed, but the Rai system is the most common in
having achieved remission) the United States. The stage is important in deter-
(C91.10-C91.12 = 3 codes of mining appropriate treatment and the prognosis. The
specificity) Rai system stratifies patients into risk groups accord-
Refer to the physician’s diagnosis for specific ing to their symptoms and assigns an average
type of lymphoid leukemia and then to the survival time to those groups. Patients usually move
current editions of the ICD-9-CM and ICD- through the risk groups during the course of their
10-CM coding manuals to ensure the greatest disease:
specificity of pathology.
• Stage 0: Patients have lymphocytosis and bone
marrow infiltration of greater than 30% blasts.
Symptoms and Signs Median survival time is 12 years.
Patients often have no symptoms when a routine CBC • Stage I: Patients have lymphadenopathy. Median
reveals lymphocytosis, thrombocytopenia, or anemia. survival time is 9 years.
Symptoms may include weight loss, fever, night • Stage II: Patients have splenomegaly and/or hepa-
sweats, extreme fatigue, splenomegaly, hepatomegaly, tomegaly. Median survival time is 6 years.
and painless swelling of cervical, supraclavicular, or • Stage III: Patients have anemia. Median survival
axillary lymph nodes that spontaneously waxes and time is 1 year.
wanes. Patients are susceptible to frequent viral and • Stage IV: Patients have thrombocytopenia. Median
fungal infections. survival time is 1 year.
rapidly progressive neoplasm of cells committed to the

Treatment myeloid line of development. Leukemic cells accumu-
Treatment often is withheld until the patient is symp- late in the bone marrow, peripheral blood, and other
tomatic and has evidence of hemolytic anemia, painful tissues.
lymphadenopathy, symptomatic organomegaly, cyto-
ICD-9-CM 205.0 (Acute promyelocytic
penia, prolonged fever, chills, or weight loss. CLL is
Code leukemia) (Requires fifth digit)
usually treated by chemotherapy. Radiation therapy ICD-10-CM C92.00 (Acute myeloblastic
can be used to reduce symptoms caused by lymphade- Code leukemia, not having
nopathy and splenomegaly. Other therapies include achieved remission)
the use of monoclonal antibodies directed against (C92.00-C92.02 = 3 codes of
B-lymphocyte surface antigens, such as rituximab specificity)
(Rituxan) (directed against CD20) or alemtuzumab C92.40 (Acute promyelocytic
(Campath) (directed against CD52). Other medica- leukemia, not having
tion therapies may include chlorambucil, pentostatin, achieved remission)
prednisone, or cladribine; transplantation is generally (C92.40-C92.42 = 3 codes of
specificity)
not an option for CLL patients, although the newly
C92.50 (Acute myelomonocytic
developed nonmyeloablative transplants show promise
leukemia, not having
as a potential treatment for CLL. achieved remission)
(C92.50-92.52 = 3 codes of
Prognosis specificity)
CLL has a variable natural course of disease. Survival Refer to the physician’s diagnosis for specific
times from diagnosis range from 2 to 20 years. The type of myeloid leukemia and then to the current
overall 5-year survival rate is 73%. Poor prognostic editions of the ICD-9-CM and ICD-10-CM coding
indicators are low platelet count and low hemoglobin manuals to ensure the greatest specificity of
level at the time of diagnosis. Certain genetic aberra- pathology.
tions have prognostic implications as well. For
example, the high risk TP53 corresponds to a 51% Symptoms and Signs
5-year overall survival while the low risk del13q14 A rapid accumulation of myeloblasts (myeloid precur-
carries a 5-year survival of 87%. During the initial sors) leads to pancytopenia and the resulting symp-
asymptomatic phase, patients often can maintain their toms of anemia, easy bleeding and bruising, and
normal lifestyle, but performance levels during the increased risk of infection. Bleeding symptoms include
terminal phase are poor, and frequent causes of death gingival bleeding, epistaxis, and menorrhagia. Weight
include systemic infection, bleeding, and cachexia. loss, fatigue, and pallor are common. Lymphadenopa-
thy and organomegaly are rare.
Prevention
No methods for prevention of CLL are known. Etiology
AML comprises 80% of acute adult and 20% of child-
Patient Teaching hood leukemia. Risk factors include a positive family
Give patients and parents of children information history for AML or other leukemias; prior therapeutic
about the chemotherapy. Help them find and contact treatment with ionizing radiation; prior aggressive
community support groups. chemotherapy treatment for Hodgkin disease, non-
Hodgkin lymphoma, ovarian cancer, or breast cancer;
chronic exposure to benzene (a toxic liquid found
Acute Myelogenous Leukemia
in gasoline, rubber cement, and cleaning solvents);
Description and cigarette smoking. About 10% to 15% of AML
Acute myelogenous leukemia (AML) (also known as cases are treatment-related (i.e., prior radiation or
acute myeloid, myelocytic, or granulocytic leukemia) is a chemotherapy). Many cases of AML occurred among
high risk cases. Complete remission is defined as

having normal peripheral blood cell counts and less
than 5% blasts in the bone marrow.
Acute promyelocytic leukemia (APL) is a form of
AML that is distinct from the other subtypes. It is
characterized by a translocation between the long
arms of chromosomes 15 and 17, t(15;17), creating a
fusion gene, PML/RAR-a. The gene product impairs
differentiation and apoptosis of promyelocytes. The
impairment can be overcome by administration of
all-trans retinoic acid (ATRA), a derivative of vitamin
• Figure 10-53 Auer rod cells. (From Wiernik, et al: Neo- A, often in conjunction with cytotoxic chemotherapy
plastic diseases of the blood, ed 3, New York, 1996, Churchill and arsenic trioxide. The patient should be monitored
Livingstone.) for signs of disseminated intravascular coagulation
(DIC) and differentiation syndrome (such as fever,
peripheral edema, respiratory distress, and renal and
hepatic dysfunction).
Japanese survivors of the atomic bomb attack in 1945
and the Chernobyl nuclear power plant in Russia Prognosis
in 1986. The overall 5-year survival rate for AML is highly
dependent on prognostic indicators and ranges from
Diagnosis 15% to 70%. Adverse prognostic indicators are
The diagnosis is suggested by the clinical picture and advanced age, history of a prior hematologic disorder,
peripheral blood smear. Half of the patients will have and poor performance status at diagnosis. Some
an Auer rod (abnormal lysosomal granules) visible cytogenetic abnormalities, such as the translocations
inside the leukemic cell (Figure 10-53). The presence t(8;21) and t(15;17), have a more favorable prognosis.
of an Auer rod is diagnostic for AML. Bone marrow The t(15;17) translocation is considered favorable
aspiration and biopsy is needed for definitive diagno- because of the excellent treatment response of APL to
sis. The bone marrow usually is hypercellular with ATRA and chemotherapy. The 5-year survival rate for
greater than 20% myeloblasts. After diagnosis, a mor- APL can approach 70% with appropriate treatment.
phologic classification and cytogenetic analysis must
be performed to determine the treatment selection. In Prevention
addition, molecular studies are done to look for spe- No methods of preventing AML are known.
cific genetic mutations that affect treatment choice
and prognosis. For the classification of AML based on Patient Teaching
morphologic cell type, the World Health Organiza- Give patients and parents of children information
tion (WHO) classification scheme is used. about the chemotherapy. Help them find and contact
community support groups.
Treatment
Routine laboratory testing (e.g., CBC, liver function
Chronic Myelogenous Leukemia
tests, and coagulation studies), chest radiograph,
ECG, and herpes simplex virus (HSV) and cytomega- Description
lovirus (CMV) serology are obtained to identify Chronic myelogenous leukemia (CML) (also known
potential complications of therapy. Chemotherapy is as chronic myeloid or myelocytic leukemia) is a slowly
the first approach to treating AML. Usually cytarabine progressing neoplasm that arises in a hematopoietic
and daunorubicin are given to induce remission. stem cell or early progenitor cell, resulting in an excess
HSCT during the first remission is recommended in of mature-appearing, but hypofunctional, neutrophils.
ICD-9-CM 205.1 (Chronic myeloid leukemia) gene product by cytogenetic analysis, fluorescence in
Code (Requires fifth digit) situ hybridization (FISH), or polymerase chain reac-
ICD-10-CM C92.10 (Chronic myeloid tion (PCR). The presence of the Philadelphia chromo-
Code leukemia, BCR/ABL-positive, some distinguishes CML from other disorders that
not having achieved remission)
may resemble CML clinically.
(C92.10-C92.22 = 6 codes of
specificity) Treatment
Refer to the physician’s diagnosis for specific
type of myeloid leukemia and then to the current Cure can only be achieved via HSCT (see the Enrich-
editions of the ICD-9-CM and ICD-10-CM coding ment box about Hematopoietic Stem Cell Transplan-
manuals to ensure the greatest specificity of tation). However, HSCT is a viable option for only
pathology. about 25% of patients. Initial treatment is started with
drugs that specifically inhibit the BCR/ABL tyrosine
Symptoms and Signs kinase, known as tyrosine kinase inhibitors (TKIs).
The disease has a triphasic course: a chronic phase, an Their use is aimed at achieving long term control of
accelerated phase, and a blast crisis. Most patients the disease, although they do not cure the disease.
are diagnosed in the chronic phase, where abnormal Many patients can obtain a complete remission on
proliferation of WBCs has begun. These patients are TKIs which can be sustained for years. Complete
usually asymptomatic at diagnosis, with the disease remission is defined as ablation of the Philadelphia
being suspected from abnormalities, such as leukocy- chromosome and its gene product.
tosis and thrombocytosis, on routine blood tests. Sple-
nomegaly is common, but symptoms are mild. In the Prognosis
accelerated phase, leukocytosis increases and neutro- The overall 5-year survival rate is 35%. Indicators of
phil differentiation becomes further impaired. The poorer prognosis include large spleen size, older age
patient may experience bone pain, fever, night sweats, of the patient, higher percentage of blast cells, and a
and other systemic symptoms. The blast crisis resem- platelet count above 700,000/μl.
bles AML and is defined by the presence of 30% or
more blasts in the peripheral blood or bone marrow. Prevention
Symptoms worsen, and new chromosomal abnormali- No methods are known to prevent CML.
ties are acquired. CML progresses to blast crisis an
average of 3 to 5 years after diagnosis and 3 to 18 Patient Teaching
months after initiation of the accelerated phase. The Give patients and parents of children information
average survival after blast crisis is 3 months. about the chemotherapy. Help them find and contact
community support groups.
Etiology
CML accounts for 15% to 20% of leukemia in adults Lymphatic Diseases
and occurs most often in those over the age of 40.
CML is almost invariably associated with an abnormal The lymphatic system is composed of lymphatic vessels,
chromosome 22, the Philadelphia chromosome. . lymphatic tissue (lymph nodes, tonsils, thymus, and
spleen), and lymph. The lymphatic vessels originate at
Diagnosis the capillary level and, along with the venous system,
The diagnosis is suggested by the clinical picture and progress to empty into the right and left subclavian
blood and bone marrow studies. A CBC reveals veins (Figure 10-54). This system has no pump. Lymph
anemia, leukocytosis, and thrombocytosis. A bone nodes, collections of lymphatic tissue, filter foreign
marrow biopsy shows hyperplasia, but the cells appear material (such as, bacteria and viruses) from the
more mature than the leukemic cells in AML. Defini- lymphatic circulation (Figure 10-55). Lymphocytes
tive diagnosis requires demonstrating the presence of are produced mainly in the lymph nodes as part of
the Philadelphia chromosome or the BCR/ABL fusion the body’s defense mechanism. The lymphatic vessels
ENRICHMENT
Hematopoietic Stem Cell Transplantation
Many patients with leukemias and lymphomas cannot low-dose radiation before transplantation of the donor
be cured with conventional chemotherapy treatments stem cells. The donor cells gradually replace the host
alone. The very large doses of chemotherapy or marrow and kill neoplastic cells by an immunologic
radiation that would be required to fully destroy all the “graft versus tumor” effect.
neoplastic cells in patients with relapsed disease or All of these transplantations carry the risk of severe
disease refractory to conventional treatment make infections and bleeding, serious damage to vital
people very sick and destroy the bone marrow. Few organs caused by initial chemotherapy and radiation
people could survive this severe treatment. therapy, and graft rejection by the host’s cells.
Hematopoietic stem cell transplantation (HSCT), Graft-versus-host disease (GVHD), an immunologic
also known as bone marrow transplantation (BMT), reaction in which the donor T lymphocytes see the
was developed to treat these people. In the traditional host tissue, especially the skin, liver, and
BMT protocol, the patient first receives high-dose gastrointestinal (GI) tract, as foreign and attack it, is
chemotherapy and/or radiation therapy to eradicate all not uncommon. Relapse may also occur and is seen
malignant cells in the body and to suppress the most often with ALL and CML transplanted during
body’s immune system so that it is less likely to blast crisis.
attack the donor marrow. Next, stem cells obtained Definitions of some terms commonly used in
from a donor’s bone marrow are infused into the conjunction with BMT are:
recipient. The stem cells find their way into the • Autologous transplant: Transplant in which the
marrow space of the recipient and restore marrow patient serves as his or her own donor for stem
functioning in 2 to 4 weeks. cells.
A newer method of HSCT has been developed in • Allogeneic transplant: Transplant in which the
recent years. Studies have shown that stem cells may donor is HLA-identical to the patient (usually a
be harvested directly from the donor’s peripheral sibling).
blood after hematopoietic growth factors have been • Haploidentical transplant: Transplant in which the
administered to the donor to mobilize stem cells from donor is a relative only half-matched to the
the marrow to the bloodstream. The stem cells are recipient (usually a parent).
collected using a pheresis machine and then infused • Human leukocyte antigen (HLA) type: The identity
into the recipient. These peripheral blood cells can of the leukocyte surface antigens, the HLAs, is
restore neutrophil and platelet production several days determined for the patient and donor to define the
faster than stem cells derived from bone marrow. degree of compatibility. The closer the match is,
Therefore this peripheral-blood, stem-cell the lower the risk of transplant-related
transplantation is preferred over the traditional HSCT complications.
for many patients. • Syngeneic transplant: Transplant in which the
An investigational approach called patient’s identical twin serves as the stem cell
nonmyeloablative transplantation, or “mini donor.
transplantation,” has shown some promise in treating • Unrelated donor/mismatched transplant:
the elderly and other patients who are unable to Transplant in which the donor does not match all
tolerate the high initial doses of chemotherapy in of the key HLA antigens. This type of transplant is
conventional BMT. It uses mild chemotherapy and associated with the greatest risk of complications.
are thin walled, and the larger vessels contain valves.

Lymphedema
Similar to the case for the venous system, the muscles
exert intermittent pressure on the vessels, causing the Description
lymph to flow by a milking action. Smooth muscle Lymphedema is an abnormal collection of lymph,
in the vessel wall contracts to aid the return of lymph usually in the extremities.
to the cardiovascular system. Swollen lymph nodes or ICD-9-CM Code 457.1 (Other lymphedema)
glands may indicate trapping of microbes during an 457.0 (Postmastectomy
infectious process. lymphedema syndrome)
Afferent lymphatic vessels
Germinal
centers
where
lymphocytes
form
Lymphatic sinus
Fibrous capsule
Efferent lymphatic vessels
• Figure 10-54 Lymph node.
ICD-10-CM Code I89.0 (Lymphedema, not

elsewhere classified)
I97.2 (Postmastectomy
lymphedema syndrome)
Symptoms and Signs

Lymphedema results in a swelling of the extremity.
Most patient experiences no pain though in severe
cases lymphedema can cause pain and limit move-
ment. The extremity becomes swollen and grossly
distended (Figure 10-56).
Patient Screening
Patients reporting unilateral limb swelling require
prompt assessment. Schedule these patients to be seen
as soon as possible or refer them to a facility where
they can be assessed quickly.
Etiology
The obstruction of the lymph vessel or node may be
inflammatory or mechanical. If lymphedema is left
untreated, the connective tissues lose their elasticity
• Figure 10-55 Lymphatic system.
and the edema becomes permanent. The lymphatic
circulation may be compromised by infections, neo-
plasms, or thrombus. Allergic reactions also may be
implicated, along with trauma or surgery involving
are applied when the affected part is elevated to com-

press the area, also encouraging lymph drainage.
Diuretics may be administered to reduce fluid volume.
Surgical intervention may be attempted to relieve a
mechanical obstruction. Antibiotics may be adminis-
tered if infection is present.
Prognosis
The prognosis varies and depends on the cause of the
lymphedema and the patient response to therapeutic
intervention. When conservative measures fail to
reduce the swelling, surgical intervention to remove
the cause of the obstruction usually has a positive
outcome. When lymphedema is left untreated, the
connective tissues can lose their elasticity, resulting in
the edema becoming permanent.
Lymphedema is not in itself life threatening, but
it carries a danger that uncontrolled infection can
develop in the affected tissue. The essentially stagnant
interstitial fluids are a breeding ground for infections
and their resulting toxins (poisons). Local defenses are
overwhelmed, and the normal systemic defense system
is not activated.
Prevention
• Figure 10-56 Lymphedema. Many cases are not preventable. Wearing of tight cloth-
ing that can restrict lymphatic flow is discouraged.
the affected part. Tight clothing that constricts the Patient Teaching
lymphatic vessels can cause temporary lymphedema. Give patients information about preventable causes
Removing the constriction usually resolves the swell- of lymphedema. Encourage postmastectomy patients
ing. Women who have had mastectomies may experi- to follow prescribed rehabilitation exercises to help
ence lymphedema in the adjacent arm. Prolonged reduce the incidence of lymphedema.
lymphedema rarely is associated with the development
of lymphosarcoma (a cancer).
Lymphangitis
Diagnosis Description
Painless swelling in an extremity suggests lymph- Lymphangitis is an inflammation of the lymph vessels.
edema. Imaging procedures, including lymphangiog-
ICD-9-CM Code 457.2
raphy and radioactive isotope studies, are means of
ICD-10-CM Code I89.1 (Lymphangitis)
confirming the diagnosis and ascertaining the site of (I89.0-I89.9 = 4 codes
obstruction. of specificity)
Treatment Symptoms and Signs

Treatment is aimed at reducing the swelling. The Lymphangitis usually is manifested by a red streak at
affected limb is elevated above the heart to encourage the site of entry of the infective organism. The redness
drainage of the lymph. Elastic bandages or stockings extends to the regional lymph node, which is swollen
and tender. Cellulitis may develop in surrounding Lymphoma

tissue. Manifestations of generalized infection, includ-
ing fever, chills, and malaise, are present. Lymphomas are malignant neoplasms that arise from
uncontrolled proliferation of the cellular components
Patient Screening of the lymph system. The dysfunctional cells may be B
Patients reporting fever, chills, and generalized malaise cells, T cells, or, rarely, both. The neoplastic lympho-
accompanied by tenderness and redness in the region cytes are migratory and can be found not only in the
of a lymph node require prompt assessment by a lymph structures, but also in the bloodstream, bone
physician. marrow, and, later in the disease, non-lymph organs
(e.g., liver or lung). Lymphomas are divided into two
Etiology main categories: Hodgkin lymphoma and the other
Bacterial invasion into the lymph vessels at the site of types that are grouped as non-Hodgkin lymphoma.
local trauma or ulceration is a frequent cause of lym- Treatment selection is determined by the cell type
phangitis. Occasionally, no portal of entry is detect- and the stage of the disease. Both kinds of lymphoma
able. The bacteria travel to the regional lymph nodes are staged using the Ann Arbor-Cotswolds staging
and stimulate inflammation. system, which considers the neoplastic involvement of
lymph structures (lymph nodes, spleen, and thymus),
Diagnosis extranodal tumor sites, and the presence or absence of
Visual inspection of the involved area and observance the systemic “B” symptoms of lymphoma (unex-
of typical systemic manifestations of bacterial invasion plained weight loss of more than 10% of body weight
are usually sufficient for diagnosis. Blood studies indi- in the past 6 months, persistent or recurrent fevers
cate leukocytosis. Final confirmation is made by cul- above 38° C during the previous month, and recur-
tures of the infected tissue. rent, drenching night sweats during the previous
month).
Treatment • Stage I: A single lymph structure or region is
Treatment includes the administration of systemic involved.
antibiotics. The affected area is elevated and rested, • Stage II: Two or more lymph structures are
and warm and wet dressings are applied locally. Surgi- involved, with the involvement being on the same
cal drainage of purulent material is indicated. side of the diaphragm.
• Stage III: Lymph regions on both sides of the dia-
Prognosis phragm are involved.
The prognosis varies depending on the amount of • Stage IV: There is widespread involvement of extra-
tissue involved and the nature of the causative organ- nodal tissue above and below the diaphragm.
ism. The patient response to antibiotics also plays a Each stage designation carries either an “A” or a
role in recovery. Surgical intervention usually contrib- “B” after the stage number, indicating whether any of
utes to a more positive outcome. the “B” symptoms are absent (A) or present (B).
Prevention
Hodgkin Lymphoma
This condition usually cannot be prevented. Good
hand washing always helps prevent any infectious Description
process. Hodgkin disease (also called Hodgkin lymphoma) is a
cancer of the body’s lymphatic system, in which the
Patient Teaching involved cells proliferate and interfere with normal
Give patients information about care of postoperative functioning by collecting in masses in various parts of
incision sites. Emphasize the importance of comply- the body. Tumors arise in the tissue of the lymph
ing with, and completing, any prescribed antibiotic nodes and spread to other lymph nodes, the spleen,
therapy. the liver, and the bone marrow.
ICD-9-CM Code 201.90 (Unspecified) weight loss. Hodgkin disease is differentiated from
ICD-10-CM C81.90 (Hodgkin lymphoma, other lymphomas by the presence in the lymphatic
Code unspecified, unspecified tissue of a Reed-Sternberg cell, a large cell with two
site) or more mirror-image nuclei, each with a single nucle-
C81.99 (Hodgkin lymphoma,
olus (Figure 10-58).
unspecified, extranodal
and solid organ sites) Patient Screening
(C81.00-C81.99 = 70 codes
of specificity) Patients reporting a painless enlargement of cervical
Hodgkin disease is coded by type. Refer to the lymph nodes, fatigue, and pruritus should be sched-
physician’s diagnosis and then to the current uled as soon as possible. When the complaints include
editions of the ICD-9-CM and ICD-10-CM coding fever, night sweats, and weight loss, prompt assess-
manuals to ensure the greatest specificity of ment by a physician is required.
pathology.
Etiology
Symptoms and Signs Hodgkin disease represents 15% of all lymphomas.
The initial symptoms of Hodgkin lymphoma are pain- There are two peaks of incidence: one in patients in
less enlargement of the lymph nodes in the neck or their 20s and the other in those over the age of 50.
mediastinum, fatigue, and pruritus (Figure 10-57). As Risk factors for the development of Hodgkin disease
the disease progresses, the patient may experience the include previous history of malignancy, prior treat-
systemic B symptoms of fever, night sweats, and ment with chemotherapy or radiation therapy, family
history of Hodgkin disease or other lymphomas,
immunosuppression, and exposure to the Epstein-
Barr virus.
Epitrochlear
and brachial
Left cervical nodes
supraclavicular
nodes Left axillary nodes
Right cervical
supraclavicular
Retroperitoneal
nodes
nodes (located
Right axillary behind the
nodes peritoneum)
Epitrochlear Mesenteric nodes
and brachial
nodes Left inguinal
Right inguinal nodes
nodes
Common
site
Popliteal nodes Uncommon
site
• Figure 10-57 Lymph node sites for Hodgkin disease. • Figure 10-58 Reed-Sternberg cell. (From Wiernik, et al:
(From Huether SE, McCance KL: Understanding pathophysi- Neoplastic diseases of the blood, ed 3, New York, 1996,
ology, ed 4, St Louis, 2008, Mosby/Elsevier.) Churchill Livingstone.)
Diagnosis Non-Hodgkin Lymphoma

A history of painless lymphadenopathy and the typical Description
symptoms of Hodgkin disease lead to an excisional Non-Hodgkin lymphoma (NHL) describes a number
lymph node biopsy for definitive diagnosis. The node of heterogeneous neoplasms of the lymphoid cells that
biopsy shows the presence of Reed-Sternberg cells. exhibit a wide variety of clinical signs and symptoms,
Immunophenotyping of the tissue for certain markers ranging from slow, indolent growth to rapidly fatal
is very helpful in making the diagnosis. Blood studies progression. Some can be cured with appropriate
indicate a mild normochromic, normocytic anemia; treatment, but for others, treatment does not prolong
neutrophilic leukocytosis; lymphopenia; and eosino- survival.
philia. The ESR is elevated, as is the serum alkaline
ICD-9-CM Code 202.8
phosphatase level. Bone marrow biopsy is performed
ICD-10-CM Code C85.80 (Other specified
only on select patients and may show abnormal cells. types of non-Hodgkin
CT scans of the chest, abdomen, and pelvis help eval- lymphoma, unspecified
uate the extent of disease. Liver and kidney function site)
tests are performed. C85.89 (Other specified
types of non-Hodgkin
Treatment lymphoma, extranodal
Combined chemoradiotherapy is preferred for the and solid organ sites)
patients in stages I and II and some patients in stage III. (C85.10-C85.99 = 40
Patients in stage IIIB and stage IV often are treated with codes of specificity)
chemotherapy alone. The preferred regimen is ABVD
(doxorubicin, bleomycin, vinblastine, dacarbazine) Symptoms and Signs
therapy. Other medication therapies may include chlo- As with Hodgkin lymphoma, symptoms include pain-
rambucil, pentostatin, prednisone or cladribine. Relapses less lymphadenopathy, fatigue, pruritus, bone pain,
usually are treated with additional chemotherapy. HSCT and GI symptoms. Other lymphatic tissue, such as
may be used to treat patients with multiple relapses. tonsils and adenoids, may be enlarged, and the patient
can experience the “B” symptoms of fever, night
Prognosis sweats, and weight loss.
Hodgkin lymphoma is one of the most treatable forms
of cancer and can be cured. The overall 5-year survival Patient Screening
rate approaches 90%. Death may result from Hodgkin As with Hodgkin lymphoma, patients reporting a
lymphoma itself, or from secondary cancers or cardio- painless enlargement of cervical lymph nodes, fatigue,
vascular disease, side effects of treatment that often are and pruritus should be scheduled as soon as possible.
not apparent until years after therapy. Important indi- When the complaints include fever, night sweats, and
cators of poor prognosis include the presence of weight loss, prompt assessment by a physician is
B symptoms, low serum albumin and hemoglobin required.
levels, male gender, age over 45 years, anemia, leuko-
cytosis, and a high ESR. Etiology
The incidence of NHL reaches a peak in preadoles-
Prevention cents, drops in incidence, and then increases again
No ways of preventing Hodgkin lymphoma are known. with increasing age. Risk factors include a personal
or family history of prior malignancy; previous treat-
Patient Teaching ment with radiation therapy, immunotherapy, or
Give patients information about chemotherapy and chemotherapy; infection with human immunodefi-
radiation therapy. Help them find and contact com- ciency virus (HIV), human T-cell lymphotropic virus
munity support groups. (HTLV), Epstein-Barr virus, or hepatitis C virus;
immunosuppression, and connective tissue disorders, necessitate starting chemotherapy or local radiation
such as lupus and rheumatoid arthritis. GI lymphoma for palliation as indolent lymphomas are generally not
may be seen in patients with Crohn disease, celiac curable with conventional therapies. The more aggres-
disease, and Helicobacter pylori-associated chronic sive lymphomas have a rapid progression but may be
gastritis. cured with appropriate treatment. They are treated
NHL is grouped into three main categories based with CHOP chemotherapy (cyclophosphamide,
on how aggressively each neoplasm in the group doxorubicin, vincristine, and prednisone) with or
behaves. The indolent lymphomas represent 35% to without rituximab and with or without radiation
40% of NHLs. The most common subtypes are follicu- therapy. Relapses may be treated by high-dose chemo-
lar lymphoma (grades I and II behave as indolent; grade therapy followed by HSCT or by chemotherapy
III behaves as aggressive), small lymphocytic lym- alone. Patients are also at increased risk for develop-
phoma, mantle cell lymphoma, and marginal zone lym- ment of second malignancies and should have peri-
phoma. They may arise from any B-cell, T-cell, or odic follow-up after treatment.
natural killer (NK)-cell line. Aggressive lymphomas
represent 50% of NHLs. The most common subtypes Prognosis
are diffuse large B-cell lymphoma and peripheral T-cell The 5-year overall survival rate is 69%. Histopathol-
lymphoma. Highly aggressive lymphomas are rarer, rep- ogy is the most important prognostic indicator, fol-
resenting 5% of all NHLs. These include Burkitt lym- lowed by patient age, presence of extranodal disease,
phoma (thought to be caused in part by the Epstein-Barr presence of “B” symptoms, and stage at the time of
virus) and adult T-cell lymphoma (caused by HTLV). diagnosis. The indolent lymphomas generally are asso-
ciated with a survival measured in years even if left
Diagnosis untreated. However, they generally are not curable
The patient evaluation must include determination of with treatment. In contrast, the aggressive lymphomas
the histologic subtype, extent of disease, and perfor- are curable but are rapidly fatal if left untreated or if
mance status of the patient because treatment and they are unresponsive to therapy.
prognosis greatly depends on this information. All
potentially involved lymphoid sites should be physi- Prevention
cally examined. Excisional biopsy of an intact, involved No methods of prevention for NHL are known.
lymph node is necessary for accurate histopathologic
identification of the disease. Immunologic, cytoge- Patient Teaching
netic, and molecular studies may be performed to Give patients information about chemotherapy and
further aid in therapeutic decisions and determination radiation therapy. Help them find and contact com-
of prognosis. Bone marrow aspiration and biopsy are munity support groups.
performed to determine the extent of disease. If the
marrow is involved, the patient is placed at stage IV.
Lab tests after diagnosis generally include CBC, Transfusion Incompatibility Reaction
peripheral blood smear, and studies of renal and liver
function. Chest radiographs and CT scans of the Description
abdomen, chest, and pelvis are performed to determine Transfusion incompatibility results when the blood or
the extent of disease. PET scan is a useful adjunct. GI blood product transfused has antibodies to the recipi-
endoscopy may detect GI involvement (seen in 10% ent’s RBCs or the recipient has antibodies to the
to 60% of patients). An MRI of the CNS may be donor’s RBCs.
performed if neurologic signs are present in the patient. ICD-9-CM 999.6 (ABO incompatibility)
Code 999.7 (Rh incompatibility
Treatment reaction)
The treatment plan for indolent lymphoma usually 999.8 (Other transfusion
involves watchful waiting until the symptoms reaction)
ICD-10-CM T80.30X(A)(D)(S) (ABO included in the discussion as long as HIPAA guide-

Code incompatibility reaction due lines are followed.
to transfusion of blood or
blood products, unspecified) Etiology
T80.40X(A)(D)(S) (Rh
ABO- and Rh-incompatible blood as well as antigens
incompatibility reaction due
that screening does not reveal cause an antigen-
to transfusion of blood or
blood products, unspecified) antibody reaction that produces hemolysis (destruc-
T80.92X(A)(D)(S) (Unspecified tion of RBCs) or agglutination (clumping of RBCs
transfusion reaction) that obstructs the flow of blood through capillaries).
Histamine and serotonin are released from mast cells
and platelets. DIC usually is triggered (see the “Dis-
Symptoms and Signs seminated Intravascular Coagulation” section) with
This hypersensitivity reaction can range from mild resulting coagulation problems (Figure 10-59).
to fatal. Most severe transfusion reactions are
incompatibility-related and are characterized by Diagnosis
hemolysis or agglutination. Other forms include bac- Any sign of chills, fever, hives, back pain, or dyspnea
terial, allergic, and circulatory-overload transfusion during the transfusion alerts the health care profes-
reactions. The severity of the reaction depends on the sional attending the patient to a possible reaction.
amount of incompatible blood that is transfused and Blood and urine specimens are examined, along with
prior transfusion reactions of the patient. the used blood, to confirm an incompatibility and the
The patient with incompatibility reaction who is presence of hemolysis or activated coagulation.
receiving the blood transfusion experiences chills,
fever, and tachycardia. The patient with a more severe Treatment
reaction has severe back pain, vomiting, diarrhea, Transfusion protocol mandates that a set of baseline
hives or rash, a substernal tightness, and dyspnea. The patient vital signs be taken before the start of a trans-
patient becomes hypotensive and progresses to a state fusion. During the first 15 minutes, the patient is
of circulatory collapse. As the condition worsens, observed closely, and assessment of vital signs is
there is bleeding from the puncture site, blood in the repeated. This monitoring of vital signs and observa-
urine, and eventually renal failure. tion of the patient continues at designated intervals
The most frequent transfusion reaction is associ- until the procedure is completed. At the first indica-
ated with WBCs or WBC remnants in donor blood. tion of any symptoms or signs of reaction, the blood
It is febrile and short lived, ceasing when the transfu- transfusion is stopped immediately. Blood and urine
sion is halted, and there is no hemolysis or allergic samples are obtained from the patient and sent to the
response. laboratory, along with the remaining untransfused
The patient experiencing an allergic reaction exhib- blood. Mild reactions are treated with antihistamines,
its hives and itching and possibly bronchial spasms and anaphylaxis is treated aggressively according to
and anaphylaxis. institutional protocol.
Patient Screening Prognosis

Patients receiving blood transfusions will be in a clinic The prognosis varies depending on the amount of
or hospital environment. The trained professionals blood infused, the cause of the reaction, and the speed
observing the patient will immediately institute inter- of the intervention.
vention and care to reverse or modify the reaction.
Those who have survived the reaction and family Prevention
members may request additional information about Prevention is the best form of treatment. Careful
the reaction. Schedule an appointment as soon as typing and crossmatching of the blood product is
possible for the discussion. Family members may be mandatory. Two attendants must compare the patient
Donor type A Recipient type A No agglutination

blood with A blood with "Anti-B"
agglutinogens agglutinins
A
Donor type B Recipient type A Agglutination Hemolysis
blood with B blood with "Anti-B"
agglutinogens agglutinins
+ +
B
• Figure 10-59 Illustration showing agglutination of red blood cells (RBCs) during blood transfusion
incompatibility reaction. (From Applegate E: The anatomy and physiology learning system, ed 4, St Louis,
2011, Saunders/Elsevier.)
information with that on the blood bag and physi- may be ecchymosis at birth or bleeding from a cir-
cian’s orders before starting the transfusion. cumcision. Joint swelling and pain suggest bleeding in
the joints.
Patient Teaching
Instruct the patient to report any untoward symptoms Patient Screening
or unusual sensations during the transfusion process When parents report that a male infant, toddler, or
immediately, especially early in the transfusion. child has experienced unexplained prolonged bleed-
ing, noticeable hematomas, easy bruising, and/or
excessive nosebleeds, the physician should promptly
Clotting Disorders assess the child. The condition often is diagnosed
during the neonatal period of hospitalization. These
Classic Hemophilia
parents may request an appointment to learn about
Description the disorder. Schedule the patient as soon as possible
Classic hemophilia is a hereditary bleeding disorder for the next available appointment.
resulting from deficiency of clotting factors.
Etiology
ICD-9-CM Code 286.0 (Congenital factor
An X-linked genetic disorder in males, hemophilia
VIII disorder)
ICD-10-CM Code D66 (Hereditary factor is transmitted by the asymptomatic carrier mother
VIII deficiency) to her son. Factor VIII, a clotting factor in the intrin-
sic clotting cascade, is functionally inactive. Any
minor trauma can initiate the bleeding episode
Symptoms and Signs (Figure 10-60).
The condition can be mild, moderate, or severe. Any
unusually prolonged bleeding episode, easy bruising, Diagnosis
hematomas, or excessive nosebleeds in a male child The diagnosis is based on the clinical picture and a
suggest hemophilia. The first sign of hemophilia thorough history. Clotting studies indicate normal
Carrier female Hemophiliac male

Hemophiliac male
Nonhemophiliac
Carrier female
Noncarrier
E10-11 Inheritance patterns in hemophilia. (From Beare:

Adult Health Nursing, ed 3, St. Louis, 1998, Mosby.)
INTRINSIC EXTRINSIC
Kallikrein PK
PTT HMWK PT
XII, XI, IX, VIII XII contact VII, X, V, II
X, V, II
IX TF
XIIa VIIa
XI XIa
IXa TF • VIIa
• X TFPI
VIIa
VIII Xa v
Xa-Va-II
THROMBIN
FIBRINOGEN FIBRIN MONOMER
XIII XIIIa
FIBRIN POLYMERS
• Figure 10-60 Illustration of clotting cascade where factor VIII is inactive. (Andreoli TE, et al: Andreoli
and Carpenter’s Cecil essentials of medicine, ed 8, Philadelphia, 2010, Saunders/Elsevier.)
platelet count, bleeding time, and prothrombin time intervention. Blood-borne infections may complicate
(PT); prolonged partial thromboplastin time (PTT); the condition and place the patient at higher risk for
and a factor VIII assay of 0% to 30%. other disease entities.
Treatment Prevention
Hemophilia cannot be cured, but treatment prevents No prevention is known for this condition. Genetic
crippling deformities. Concentrated factor VIII (anti- counseling may help in exploring the probability of
hemophilic factor [AHF]) is administered to stop the the disorder being transferred to offspring.
bleeding. Transfusions of whole blood may be neces-
sary. The patients are encouraged to avoid situations Patient Teaching
that initiate bleeding episodes. Cases have been docu- Give parents information about X-linked genetic
mented of HIV infection resulting from transfusion disorders.
with contaminated or infected blood products before
1987 when blood supplies began being treated to
Disseminated Intravascular Coagulation
prevent viral transmission.
Description
Prognosis DIC is a condition of simultaneous hemorrhage and
No cure for hemophilia is known. Transfusions thrombosis. It is a syndrome occurring secondary to
of blood or blood products may be a lifelong other diseases or dramatic events.
ENRICHMENT Patient Screening

Most of these individuals will be in an in-patient
Lupus Anticoagulant
facility, and immediate response is indicated. If they
The patient with lupus anticoagulant (LA) usually
suffer the condition at home after a major precipi-
does not have the lupus erythematosus; however, tating event, they must be entered into the emer-
some may progress to the disease with joint pain, gency medical system for prompt assessment. Family
dermatologic problems, or renal involvement. members requesting an appointment for information
Therefore the lupus portion of the name is often after the event should be scheduled as soon as possible
considered as a misnomer. The anticoagulant portion
of the name is also a misnomer as the condition
according to HIPAA guidelines.
actually does not involve anticoagulant activity but
has that of a coagulant with formation of clots. Etiology
Many individuals with LA are asymptomatic. Thrombin activates the production of fibrin, causing
Symptoms that may occur are abnormal bleeding of clots to form where they are not needed (i.e., in the
nose or gums, atypical bruising, abnormal menstrual
cycles, multiple miscarriages, blood clots in the legs
microcirculation). The thrombin also causes platelet
(deep vein thrombosis) or in lungs (pulmonary aggregation, forming more clots. In addition, the
embolism), strokes, and heart attacks. fibrinolytic system is activated by the presence of
Blood tests are performed for the anticoagulant thrombin in the plasma; thrombin causes excessive
antibody. When a positive result is obtained, tests fibrinolysis and additional bleeding. Predisposing
are repeated. Some individuals with positive test
results do not require any treatment. Others require
factors include hypotension, hypoxemia, acidosis, and
ongoing treatment with anticoagulant medications, stasis of capillary blood. Any of these factors may be
including heparin or warfarin (Coumadin). Some may the result of the aforementioned major precipitating
be instructed to take aspirin. Many require events (Figure 10-61).
anticoagulant drug therapy along with routine blood
tests for their lifetime. These individuals are at risk Diagnosis
for blood clots that may lead to pulmonary
embolism, stroke, or heart attack. The diagnosis is based on the clinical picture; a thor-
ough history, including a probable precipitating event;
and laboratory studies. Platelet count and fibrinogen
levels are reduced, whereas PT is prolonged.
ICD-9-CM Code 286.6
ICD-10-CM Code D65 (Disseminated Treatment
intravascular coagulation Administration of IV heparin inhibits the formation of
[defibrination syndrome]) additional microthrombi and prevents the aggregation
of platelets. Platelet replacement and plasma-clotting
factors are administered when serious hemorrhage is
greatest specificity of pathology. present. The condition is life threatening and often fatal.
Prognosis
Symptoms and Signs The prognosis is guarded.
Oozing of blood from needle puncture sites, mucous
membranes, or incisions may be noted, as may bleed- Prevention
ing in the form of purpura, wound hematomas, or Because this condition is a sequela to a major systemic
petechiae. Hematemesis, hematuria, and bloody insult, prevention is unlikely.
stools may be present. The patient is weak, reports
headaches, and experiences air hunger and tachycar- Patient Teaching
dia. DIC follows a major event, such as obstetric If the patient survives the event, teaching may include
complications, septicemia, trauma, burns, hypother- an explanation of the incident. Help family members
mia, and extensive tissue destruction. to find and contact support groups in the community.
Massive tissue Endothelial

Sepsis
destruction injury
Release of tissue
factor
Platelet
aggregation
Widespread
microvascular
thrombosis
Activation of plasmin
Microangiopathic Vascular
hemolytic anemia occlusion
Consumption of
Proteolysis of Ischemic tissue
Fibrinolysis clotting factors
clotting factors damage
and platelets
Fibrin split products
Inhibition of thrombin, platelet Bleeding

aggregation, and fibrin polymerization
E10-12 Pathophysiology of disseminated intravascular coagulation. (From Kumar: Robbins Basic Pathol-
ogy, ed 9, Philadelphia, 2013, Saunders.)
Massive tissue Endothelial

destruction Sepsis injury
Release of tissue
factor Platelet
aggregation
Widespread
microvascular
thrombosis
Activation of
plasmin
Microangiopathic Vascular Consumption of
hemolytic anemia occlusion clotting factors
and platelets
Fibrinolysis Proteolysis of Ischemic tissue

clotting factors damage
Fibrin split
products
Bleeding
Inhibition of thrombin, platelet
aggregation, and fibrin polymerization
• Figure 10-61 Pathophysiology of disseminated intravascular coagulation (DIC). (From Kumar V, Cotran
R, Robbins S: Robbins basic pathology, ed 8, Philadelphia, 2008, Saunders/Elsevier.)
Review Challenge
Answer the following questions: 11. To what does cardiomyopathy refer, and how is
1. What are the common symptoms of cardiovascu- it treated?
lar disease? 12. How does endocarditis affect the cardiac valves?
2. How would you describe the pathology in coro- 13. Why is rheumatic fever described as a systemic
nary artery disease (CAD)? disease? What infection often precedes it?
3. How is the patient likely to describe the pain in 14. What is the relationship between rheumatic fever
angina pectoris? and valvular heart disease?
4. How do the symptoms of myocardial infarction 15. What are the sources of cardiac arrhythmias?
(MI) differ from those of angina pectoris? How are they diagnosed?
5. Why is angioplasty used for MI? 16. What are some of the treatment options for
6. What conditions can cause cardiac arrest? What cardiac arrhythmias?
is the time frame in which cardiopulmonary 17. Why is shock considered a life-threatening emer-
resuscitation (CPR) may be successfully gency? What are the signs and symptoms?
initiated? 18. What is cardiac tamponade?
7. What are the diagnostic criteria for essential 19. What is the relationship between venous throm-
hypertension? bosis and pulmonary emboli?
8. What happens when the pumping action of the 20. What is the difference between arteriosclerosis
heart is inadequate? How can congestive heart and atherosclerosis? Why are both conditions a
failure (CHF) be treated? health threat?
9. Which conditions may lead to cor pulmonale? 21. How might an aneurysm be detected? When are
10. How is pulmonary edema treated? they a serious threat?
22. What is the relationship of phlebitis to thrombo- 30. Which leukemia is considered more a disease of
phlebitis? Why should the clinician avoid massage later life?
of the affected area? 31. How does lymphedema differ from lymphangitis?
23. What medical intervention is available for severe 32. Which lymphoma is characterized by the pres-
varicose veins? ence of Reed-Sternberg cells in lymphatic tissue?
24. Which conditions may precipitate an episode of 33. What symptoms does a patient experience with a
Raynaud disease? transfusion incompatibility reaction?
25. What are the presenting symptoms of anemia? 34. How is classic hemophilia treated?
26. What are some possible causes of anemia? 35. Referring to the American Heart Association’s
27. How are the following anemias differentiated: website, list the American Heart Association
aplastic, sickle cell, hemolytic, iron-deficiency, (AHA) recommended dietary guidelines for a
hemorrhagic, and pernicious? healthy diet that is suggested as a weapon to fight
28. What are some usual causes of agranulocytosis? cardiovascular disease.
29. What are typical symptoms in all types of
leukemia?
Real-Life Challenge: Essential Hypertension

A 46-year-old man reports intermittent headaches and hypertension, and his two sisters are alive and well.
light-headedness for about 3 months. In the past 2 The patient smokes one pack of cigarettes a day and
weeks, he has had several episodes of epistaxis. Vital has for 30 years.
signs are temperature, 98.6° F; pulse, 106; respira- The patient is advised to stop smoking or at least
tions, 20; and blood pressure, 168/98. His weight is cut down on the number of cigarettes smoked per day.
265 lbs., and his height is 5 feet, 11 inches. In addition, he is advised to begin a weight-reduction
The history reveals that the patient has a highly and exercise program and to reduce sodium intake. If
stressful job requiring extensive travel with overnight possible, he also should reduce stressful situations.
stays. He regards himself as a high achiever. A family Medications ordered are a beta-blocker, atenolol, and
history discloses that his father died at 53 years of age an ACE inhibitor, ramipril. The patient is instructed
of a CVA and that his mother at 69 years of age has to return in 1 week for reevaluation. Essential hyper-
CHF and hypertension. His brother has a history of tension is a suspected diagnosis.
Questions
1. What factors contribute to the symptoms and signs 5. What is the significance of the family history?
of essential hypertension that this patient is 6. What is the action of CCBs in the treatment of
experiencing? essential hypertension?
2. Why would his onset of symptoms be considered 7. If essential hypertension is not controlled, what
insidious? complications may evolve from the condition?
3. Why could the diagnosis of essential hypertension 8. How important was the symptom of epistaxis in
not be confirmed on the initial encounter? the diagnosis?
4. Why might a diuretic be ordered?
Real-Life Challenge: Anemia

A 32-year-old woman reports feeling tired all the time, headedness. She appears quite pale. Vital signs are:
having an excessively fast heartbeat, having difficulty temperature, 98.4° F; pulse, 118; respirations, 26; and
catching her breath, and experiencing some light- blood pressure, 100/69.
The patient is married, has three children ages 7 is below normal and normocytic; hemoglobin is 9,
years, 4 years, and 6 months, and is employed outside hematocrit is 27, and WBC and platelet counts are
the home. She says she can scarcely get out of bed in normal. The patient is diagnosed with iron-deficiency
the morning and can barely make it through the day. anemia and encouraged to rest and to increase her
She has had four episodes of syncope in the past week. dietary intake of red meat, liver, and egg yolks. Sup-
Menstrual periods have been regular but heavy. She plementary iron is prescribed in the form of ferrous
denies experiencing any observable blood in urine or sulfate tablets, and the patient is instructed to return
stools. A CBC with differential is ordered. The RBC to the office in 1 week for follow up.
Questions
1. What might have been the cause of the iron- 6. What might blood tests show if this was sickle cell
deficiency anemia in this patient? anemia?
2. What causes RBCs to be microcytic? Macrocytic? 7. Compare hemolytic anemia with iron-deficiency
3. What is the importance of the absence of blood in anemia.
the stool and urine? 8. As the hemoglobin improves, what changes might
4. What might be the connection with the previous be expected in the vital signs?
pregnancy?
5. What type of food in addition to the red meat,
liver, and egg yolks would help increase the
hemoglobin?
Real-Life Challenge: Abdominal Aortic Aneurysms

A 75-year-old female diabetic has been diagnosed with the aneurysm had started to increase in size. The
two small abdominal aortic aneurysms. She also has a course of watchful waiting was to continue. The
history of elevated blood pressure, 150/90. Lisinopril patient was instructed to monitor her blood pressure
has been prescribed to maintain an acceptable level. (BP) closely and not to lift anything weighing more
The physician suggested that the course of treatment than 10 pounds. Additionally, she was advised not to
could be watchful waiting. A surgeon had been con- stretch or reach out.
sulted and after reviewing lab values and a CT scan The surgeon preferred to schedule a procedure to
of the abdomen and abdominal aorta, suggested not install a stent into the abdominal aorta. The patient
pursuing a surgical course at the time of the tests. had begun to experience severe muscle spasms in the
The patient was scheduled to be followed up every muscles of the lower legs. She also was unsure about
3 months. The first 3-month exam revealed no signifi- the proposed procedure and asked what would happen
cant change. The second 3-month exam showed that if she did not have the procedure.
Questions
1. What might be the cause of the aneurysms? 5. Why would the patient be instructed not to lift or
2. Why is controlling the blood pressure important? stretch?
3. Why would the surgeon want to adopt a wait and 6. What would be the outcome if the aneurysm began
see attitude at first? to leak?
4. What is the significance of the cramping in the 7. What would be the chances of survival if the aneu-
legs? rysm ruptured?
Internet Assignments
1. Research suggested treatment interventions for 3. Research the incidence of hypertension at the AHA
CHF from the AHA. In particular, research and website. Record the ages and genders of patients
list drug interventions and patient teaching about and the seriousness of their condition. Verify and
administration of these drugs. record any drug therapy suggested.
2. Research suggested treatment interventions for 4. Research the incidence of hemophilia in the U.S.
cardiac arrhythmias from the AHA. Use the population in the past 10 years at the National
arrhythmia chart in the chapter to identify Hemophilia Association website. Also research sug-
the arrhythmias that are considered potentially gested drug treatments along with side effects.
lethal arrhythmias. Record your findings from the
website about treatment interventions.
Critical Thinking
1. As a review of the anatomy of the heart, list the 13. Discuss causative agents, including infections in
four chambers and the four valves. the body that may be the cause of myocarditis
2. Identify the structures of the conduction system. and/or endocarditis.
3. After reviewing the American Heart Association’s 14. Discuss the psychological effects of an asymptom-
website, discuss the AHA Healthy Heart atic patient being diagnosed with mitral valve
Recommendations. prolapse.
4. Locate AEDs in your educational or work facility, 15. Explain what you think arrhythmias might feel
and describe how you would use the AED. like, including both atrial and ventricular types.
Discuss situations when you would use an AED. 16. Research and report on intervention for mitral
5. List and discuss the dangers of malignant stenosis.
hypertension. 17. Explain the importance of diagnosis and treat-
6. Discuss the importance of compliance with fol- ment for strep throat.
lowing the prescribed drug regimen in treating 18. What is the importance of completing the anti-
hypertension and malignant hypertension. biotic regimen prescribed for treatment of strep
7. Referring to Figure 10-22, review the course of throat?
CHF and attempt to indicate (where possible) 19. Patients with pacemakers usually have to wait
intervention that may offer some relief to the until the battery reaches “end of service” before
condition. the battery or pacemaker can be replaced. Discuss
8. Discuss the feelings that patients may experience the psychological aspects of waiting for the “end
as CHF progresses, increasing difficulty breathing. of service” message to be obtained.
9. How would you explain the differences between 20. Explain some of the precautionary measures that
cor pulmonale and CHF? might be taken by travelers or those with seden-
10. Discuss the feelings of patients experiencing tary lifestyles to avoid developing a thrombosis in
dyspnea. the legs.
11. A patient is experiencing the symptoms of cardio- 21. Discuss the psychological effects of an individual
myopathy. Discuss his/her response to dyspnea, being diagnosed with an aortic abdominal aneu-
fatigue, tachycardia, palpitations, and occasional rysm when a “watch and wait” attitude is decided
chest pain. versus surgical intervention. Research various
12. What infectious processes in the body might be methods of treatment, and then discuss treatment
the cause of pericarditis? options.
22. List the components that make up blood. 25. What might be the psychological effects of post-
23. List various types of anemias, and identify their mastectomy lymphedema?
causes. 26. Discuss hematopoietic stem cell transplantation
24. Discuss the effects of diagnosis of leukemia on the (HSCT).
patient and family, his/her workplace, and his/her
community.
Prepare to discuss Critical Thinking case study exercises for this chapter that are posted on Evolve.

Circulatory System

Uploaded by

Document Information

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Circulatory System

Uploaded by

Copyright:

Available Formats

10

Diseases and Conditions of the

through the pulmonary valve and, with ventricular

Left common carotid artery

Left subclavian artery

Right pulmonary veins Great cardiac vein

Auricle of right atrium Circumflex artery

Anterior interventricular branches of

Left subclavian artery Brachiocephalic trunk

Superior vena cava

Left pulmonary veins Right pulmonary veins

Auricle of left atrium

Inferior vena cava

Posterior artery and Posterior interventricular branch of

• Figure 10-3 Circulation through the body.

1. DIASTOLE 2. DIASTOLE 3. SYSTOLE BEGINS

6. DIASTOLE 5. SYSTOLE 4. SYSTOLE

rhythmically on their own and characteristically are

• Figure 10-7 Coronary arteries.

A. Fatty streak B. Plaque

Small lumen Total occlusion

C. Formation of thrombus D. Complications

Total occlusion CEREBROVASCULAR

E10-1 Right-sided cardiac catheterization. (From Ignatavi-

1. Partially obstructed artery A

2. Less blood flow here

Pulmonary Left atrium

Prevention Patient Teaching

Catheter in Balloon Arterial

ICD-10-CM I21.0 (fifth digit required) (ST

Left of sternum Mid-chest, Mid-chest

Upper abdomen Chest, neck, Center of lower

Inside right arm Between

• Figure 10-17 Coronary bypass graft. (From Mosby’s dic-

Implanted • Figure 10-19 Photo of an automated external defibrillator

Treatment Patient Teaching

final outcome of the event include the general health

ENRICHMENT few, if any, symptoms until permanent damage has

Decreased blood flow to kidney

Increased work for the heart

Damage to cerebral Damage to

to increase renal blood flow), ARBs, vasodilators (to

lives. Give patients and families dietary information

Lymphatic and Blood Disorders

Congestive Heart Failure

Symptoms and Signs

DECREASED CARDIAC OUTPUT

Diuretics Stimulation of aldosterone secretion

Increased blood volume

Increased work for heart

HEART MUSCLE (LEFT VENTRICLE) WEAKENS

LEFT VENTRICLE DOES NOT FULLY EMPTY

INCREASED RESISTANCE FOR RIGHT VENTRICLE

RIGHT VENTRICLE WEAKENS AND CANNOT FULLY EMPTY

RIGHT- BLOOD BACKS UP IN SYSTEMIC CIRCULATION

Treatment ICD-9-CM Code 416.9 (Chronic, NOS)

4. Backup of blood into

L 2. Decreased cardiac output

Left-sided congestive heart failure Means less

6. Very high venous pressure

3. Decreased renal blood flow

Right-sided congestive heart failure

and fibrotic pulmonary lesions, and the primary Pulmonary Edema

Ascites and edema