1

SEMINAR
ON

CARDIOMYOPAT
HY

SUBMITTED TO SUBMITTED BY
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SUBMITTED ON

CENTRAL OBJECTIVES

By the end of the class, the students acquire knowledge regarding nursing
process and apply their skills while giving care to patients in the health settings.

SPECIFIC OBJECTIVES

define cardiomyopathy

explain the incidence

list down the etiology and risk factors

classify the types of cardiomyopathy

explain dilated cardiomyopathy

describe hypertrophic cardiomyopathy

explain restrictive cardiomyopathy

discuss arrhythmogenic right ventricular cardiomyopathy

explain unclassified cardiomyopathy

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SL.NO CONTENTS PAGE NO.

1 INTRODUCTION 4

2 DEFINITION 4

3 INCIDENCE 4

4 ETIOLOGY 5

5 CLASSIFICATION

6 DIALATED CARDIOMYOPATHY

7 HYPERTROPHIC CARDIOMYOPATHY

8 RESTRICTED CARDIOMYOPATHY
ARRHYTHMOGENIC RIGHT
9 VENTRICULAR CARDIOMYOPATHY
UNCLASSIFIED CARDIOMYOPATHIES
10

11 CONCLUSION
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12 BIBLIOGRAPHY

CARDIOMYOPATHY

INTRODUCTION

‘A diverse group of conditions whose final, common pathway is myocardial

dysfunction.’ In the past, the cardiomyopathies have been described as heart muscle
disease of unknown cause and were differentiated from cardiomyopathies which
developed from specific disease of the heart muscle.

The middle layer of the heart wall that contains cardiac muscle (myocardium)
weakens and stretches, causing the heart to lose its pumping strength and become
enlarged. Cardiomyopathy is an irreversible primary disease of the heart muscle.
Cardiomyopathy affects the myocardial layer of the heart, but it can also affect the
endocardial, sub endocardial and pericardial layers.

DEFINITION

 Cardiomyopathy (CMP) constitutes a group of diseases that directly affect the

structural or functional ability of the myocardium.
 Cardiomyopathy is the measurable deterioration of the function of the
myocardium usually leading to heart failure.
 Cardiomyopathy is a weakening of the heart muscle or another problem with the
heart muscle.
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 Cardiomyopathies is defined as heart muscle disease of unknown cause and is

characterized by chamber dilation, wall thickening (hypertrophy), decreased
contractility, and conduction disturbances

INCIDENCE
The incidence of cardiomyopathy varies according to type. Cardiomyopathy causes
more than 27,000 deaths each year in the United States (American Heart Association,
2001). The mortality rate is highest for African Americans and the elderly (American
Heart Association, 2001).

ETIOLOGY
Common causes of cardiomyopathy include:
• Long-term high blood pressure
• Heart valve problems
• Heart tissue damage from a previous heart attack
• Chronic rapid heart rate
• Metabolic disorders, such as thyroid disease or diabetes
• Nutritional deficiencies of essential vitamins or minerals, such as thiamin (vitamin
b-1), selenium, calcium and magnesium
• Pregnancy
• Excessive use of alcohol over many years
• Abuse of cocaine or antidepressant medications, such as tricyclic antidepressants
• Use of some chemotherapy drugs to treat cancer
• Certain viral infections, which may injure the heart and trigger cardiomyopathy
• Iron buildup in your heart muscle (hemochromatosis)
• Genetic conditions

CLASSIFICATION

Two fundamental forms of cardiomyopathy are recognized: (1) a primary type,

consisting of heart muscle disease predominantly involving the myocardium and/or of
unknown cause; and (2) a secondary type, consisting of myocardial disease of known
cause or associated with a systemic disease such as amyloidosis or chronic alcohol use.
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The World Health Organization classified three types of cardiomyopathy according to

anatomic and pathophysiologic characteristics: dilated cardiomyopathy (DCM),
hypertrophic cardiomyopathy (HCM), restrictive or constrictive cardiomyopathy,
arrhythmogenic right ventricular cardiomyopathy (ARVC), and unclassified
cardiomyopathy.
The pathophysiology of all cardiomyopathies is a series of progressive events that
culminate in impaired cardiac output. Decreased stroke volume stimulates the
sympathetic nervous system and the renin angiotensin-aldosterone response, resulting
in increased systemic vascular resistance and increased sodium and fluid retention,
which places an increased workload on the heart.

DILATED CARDIOMYOPATHY

It is a condition in which the heart becomes weak and the chambers get large. As a
result heart cannot pump enough blood out to the body. The decreased heart function
can affect the lungs, liver, and other body systems.

INCIDENCE

Dilated cardiomyopathy is the most common type of CMP, with a prevalence of 5 to 8

cases per 100,000 people in the United States. It causes HF in 25% to 40% of cases,
occurs more frequently in middle-aged African Americans and men, and has a genetic
link in 30% of cases.

ETIOLOGY

Primary -Idiopathic

Genetic familial: 25-30% of patients have familial form, with most mutations affecting
genes encoding cytoskeletal proteins, while some affect other proteins involved in
contraction.

 Autosomal dominant inherited patterns

 Friedrich’s ataxia
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Secondary:

 Electrolyte abnormalities
 Endocrine abnormalities
 Hypertension
 Infectious causes (adenovirus, varicella zoster, hepatitis c)
 Inflammatory (collagen vascular disease, Sarcoidosis, peripartum
cardiomyopathy)
 Infiltrative diseases
 Ischemia
 Neuromuscular diseases
 Nutritional abnormalities
 Rheumatologic diseases
 Tachyarrhythmia
 Toxins Valvular heart disease
 Irradiation
 Cocaine/amphetamines
 Pregnancy: it occurs late in gestation or several weeks to months postpartum as a
peripartum cardiomyopathy. It is reversible in half of the cases.

Pathophysiology

In dilated cardiomyopathy usually both the left and right ventricles dilate, the
myocardial fibers degenerate, and a fibrotic tissue replaces viable tissue. Fibrotic tissue
is not pliable; which leads to reduced contractility and decreased stroke volume and low
cardiac output, with a compensatory increase in heart rate. These changes eventually
lead to heart failure accompanied by lethal ventricular dysrhythmias. The combination
of ventricular dilation and ineffective myocardial contractility also increases the risk of
blood pooling within the heart and subsequent blood pooling.

Clinical Manifestations

The signs and symptoms of dilated CMP may develop acutely after an infectious process
or insidiously over a period of time. Most people eventually develop heart failure.
Pulmonary congestion and/or low cardiac output are the major symptoms. . Symptoms
can include decreased exercise capacity, fatigue, and dyspnea at rest, paroxysmal
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nocturnal dyspnea, and orthopnea. As the disease progresses the patient may
experience dry cough, palpitations, abdominal bloating, nausea, vomiting, and anorexia.
Signs can include an irregular heart rate with an abnormal S 3 and/or S4, tachycardia or
bradycardia, pulmonary crackles, edema, weak peripheral pulses, pallor, hepatomegaly,
and jugular venous distention. Heart murmurs and dysrhythmias are common.
Decreased blood flow through an enlarged heart promotes stasis and blood clot
formation, and may lead to systemic embolization.

 Cardiomegaly-common symptom
 Left and right ventricular congestion(dyspnea at rest, inspiratory crepitation in
the lungs, pleural effusions, expiratory wheezing)
 Right heart failure( peripheral edema, ascites, hepatomegaly, raised JVP,)
 Low cardiac output( poor peripheral perfusion, reduced systolic BP)
 Murmurs( mitral regurgitation, tricuspid regurgitation, left bundle branch block,
systolic murmurs)

DIAGNOSTIC STUDIES

 The diagnosis of dilated CMP is made on the basis of the patient's history and by
ruling out other conditions that cause HF.
 ECG
The ECG may be normal, but can also show diffuse nonspecific ST segment
and T wave changes. Sinus tachycardia may be observed in some cases, which
can be reversed if incessant tachy arrhythmias are slowed down.
 Chest roentgenogram
Demonstrates enlargement of the cardiac silhouette due to LV dilatation,
although generalized cardiomegaly is often seen. The lung fields may
demonstrate pulmonary vascular redistribution and interstitial or, in
advanced cases, alveolar edema
 Two-dimensional and Doppler echocardiography
Echocardiography is essential noninvasive investigation. It is useful in
detecting anatomical and functional abnormalities of the heart. The two
dimensional echocardiogram characteristically reveals a dilated, poorly
contractile left ventricle. The diagnostic features are increased LV end
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diastolic dimensions and severely impaired global contractile function, with LV

fractional shortening of less than 25%.
 Chest x ray
This demonstrates biventricular enlargement and cardiomegaly.
 Radionuclide investigations
Mitigated acquisition can be used to evaluate left ventricular function and
ejection fraction, when echocardiography imaging is inadequate. A
technetium-99m labeled non radiopharmaceutical is injected into the patient
and attaches to hemoglobin in the red blood cells. Anterior, left anterior
oblique and left lateral ECG gated images are then acquired. The resulting
images depict the blood flow in the ventricle during systole and diastole,
allowing very accurate measurement of the ejection fraction. Abnormal
ventricular wall motion can also be identified.

 VO2 max exercise test

This test measures peak exercise oxygen consumed per minute, expressed
as either an absolute value or as liters per kilogram of body weight per
minute. If this is less than 10 ml/kg/min the patient will be considered for
transplantation.
 Blood tests
All patients should undergo routine biochemical blood screening to
identify or exclude the specific causes of dilated cardiomyopathy. Other
tests may include norepinephrine levels, creatine kinase isoforms
MB2/MB1 and cardiac specific antibodies.
 Cardiac catheterization
Patients undergo catheterization to exclude CHD. Right and left heart
catheterization can provide prognostic information, especially prior to
cardiac transplantation. It can also monitor clinical treatment.
Catheterization in pediatric dilated cardiomyopathy can differentiate
primary heart muscle disease from congenital coronary anomalies.
 Transvenous Endomyocardial biopsy
It is usually not necessary in idiopathic or familial DCM; however, it may be
helpful in the recognition of secondary cardiomyopathies, such as amyloidosis
and acute myocarditis.
Management
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Medical management
Fifty percent of all deaths in patients with DCM occur suddenly. A 5-year mortality rate
of 50% has been reported for DCM of various etiologies with ejection fractions below
50%. Ventricular arrhythmias are possibly the major cause, but clots and hemorrhages
secondary to treatment or bradyarrhythmias may also contribute to deaths in DCM.
 Treat the underlying cause
If the etiology of the DCM can be determined, treatment focuses on
eliminating the cause. Otherwise, general principles of medical care include
maximizing ventricular function and exercise performance, reducing associated
risks, and, late in the disease, consideration for cardiac transplantation. The
suppression of premature ventricular contractions may be associated with
improvement of left ventricular function in patients with presumed idiopathic
DCM. Medications such as digoxin and dobutamine are administered to increase
contractility. Vasodilators are given to decrease afterload and therefore left
ventricular work, and increase CO. Heparin and warfarin for long-term
anticoagulant therapy, are given to prevent clot formation in the dilated left
ventricle and if atrial fibrillation is present. Fluid and sodium restriction, along
with administration of diuretics, maintain fluid balance. Daily weights assist in
monitoring fluid status.

Pharmacological management

 Angiotensin-converting enzyme (ACE) inhibitors. ACE inhibitors are a type of

vasodilator, a drug that widens or dilates blood vessels to lower blood pressure,
improve blood flow and decrease the workload on the heart. Examples include enalapril
(Vasotec), lisinopril (Prinivil, Zestril) and captopril (Capoten).

ACE inhibitors cause an irritating cough in some people. It may be best to put up with
the cough, if patient can. Discuss the side effect with the doctor. Switching to another
ACE inhibitor or an angiotensin II receptor blocker may relieve the cough.

 Angiotensin II receptor blockers. These drugs, which include losartan (Cozaar)

and valsartan (Diovan), have many of the beneficial effects of ACE inhibitors, but they
don't cause a persistent cough. They may be an alternative for people who can't
tolerate ACE inhibitors.
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 Beta blockers. A beta blocker slows the heart rate, reduces blood pressure and
prevents some the harmful effects of stress hormones, substances produced by our
body that can make heart failure worse and can trigger abnormal heart rhythms. Beta
blockers may reduce signs and symptoms of heart failure and improve heart function.
Examples of beta blockers include carvedilol (Coreg), metoprolol (Toprol XL) and
bisoprolol (Zebeta).
 Diuretics. Often called water pills, diuretics make the patient urinate more
frequently and keep fluid from collecting in our body. The drugs also decrease fluid in
our lungs, so the patient can breathe more easily. Commonly prescribed diuretics for
heart failure include bumetanide (Bumex) and furosemide (Lasix).

Because some diuretics make our body lose potassium and magnesium, our doctor may
also prescribe supplements of these minerals. If taking a diuretic, our doctor will likely
monitor levels of potassium and magnesium in our blood through regular blood tests.

 Aldosterone antagonists. These drugs include spironolactone (Aldactone) and

eplerenone (Inspra). These medications are diuretics, but they don't reduce potassium
levels as much as some other diuretics do. They also may help the heart work better,
may reverse scarring of the heart and may help people with severe heart failure live
longer. Unlike some other diuretics, spironolactone can raise the level of potassium in
our blood to dangerous levels.
 Digoxin (Lanoxin). This drug, also referred to as digitalis, increases the strength of
our heart muscle contractions. It also tends to slow the heartbeat. Digoxin reduces heart
failure symptoms and improves our ability to live with dilated cardiomyopathy.
 Blood thinning medications. The doctor may prescribe drugs that thin the blood
(anticoagulants). These medications help prevent blood clots. Examples include aspirin
and warfarin (Coumadin).
Devices
Implantable devices used to treat dilated cardiomyopathy include:

 Biventricular pacemakers, which use electrical shocks to coordinate the actions of

the left and right ventricle.
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 Implantable cardioverter-defibrillators (ICDs), which monitor heart rhythm and

deliver electrical shocks when needed to control abnormal, rapid heartbeats, including
those that cause the heart to stop. They can also function as pacemakers.
 Heart pumps (left ventricular assist devices or LVADs). These mechanical devices
are implanted into the abdomen or chest and attached to a weakened heart to help it
pump.

 Heart transplantation

If medications fail, patient can be posted for heart transplantation.

HYPERTROPHIC CARDIOMYOPATHY

DEFINITION

Hypertrophic cardiomyopathy (HCM), also known as idiopathic hypertrophic subaortic

stenosis, is a disorder of cardiac muscle characterized by diastolic dysfunction and
hypertrophy without dilation. It is a condition in which the heart muscle becomes thick.
The thickening makes it harder for blood to leave the heart.

PREVALENCE
Several epidemiological studies have reported the prevalence of the HCM phenotype as
about 0.2 percent in the general population (e.g., 1:500), inferring that there are
500,000 people with this disease in the United States.
HCM occurs less commonly than dilated CMP, and is more common in men ages 30 to
40, than in women. In one study, HCM occurred more frequently in young African
American male athletes compared to young white male athletes.
ETIOLOGY AND RISK FACTORS
It is usually caused by gene mutations. These mutations cause the heart muscle to grow
abnormally thick. The heart muscle cells become jumbled known as myofiber disarray.

PATHOPHYSIOLOGY

The four main characteristics of HCM are

(1) Massive ventricular hypertrophy;

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(2) Rapid, forceful contraction of the left ventricle;

(3) Impaired relaxation (diastole); and

(4) Obstruction to aortic outflow (not present in all patients).

Ventricular hypertrophy is associated with a thickened intraventricular septum

and ventricular wall.

The disproportionate thickening of the interventricular septum in hypertrophic

cardiomyopathy leads to wall rigidity and thereby increases the resistance to blood flow
from the left atrium. There is also obstruction of the left ventricular outflow. In its
severest form, the left ventricular wall reaches tremendous dimensions and encroaches
on the left ventricular chamber, which becomes small and elongated. Septal
hypertrophy obstructs the left ventricular outflow tract during systole. Frequently
diastolic dysfunction in the form of stiffness of the left ventricle occurs during diastolic
filling. This stiffness raises left ventricular end-diastolic pressure, which eventually
results in elevation of left atrial, pulmonary venous and pulmonary capillary pressures.
CLINICAL FEATURES
 Hypertrophic cardiomyopathy may present in sudden death with no prior
indication of the disease, or it can evolve with increasing symptoms.
 Reduced effort tolerance
 Syncope
 A systolic murmur heard at the lower left sternal border and apex may be the only
physical sign of HCM. The murmur may occur with or without obstruction, and
usually is of a higher grade (III to IV/VI) if there is LVOTO.
 Dyspnea is the most common symptom and appears to be related to the elevated
left ventricular end-diastolic pressure.
 Fatigue, chest pain, dizziness, syncope, palpitations, CHF, and sudden death can
present at any age.
 Angina is common and seems to be related to myocardial ischemia due to the
combination of LVOTO and diminished myocardial blood flow.
 A heavy apical pulse and S4 are also common findings, reflecting atrial systole into a
noncompliant and stiff left ventricle.
 The carotid pulse in patients with HCM is characteristically brisk and bifid.
DIAGNOSTIC EVALUATION
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The aim of the investigations in HCM is to establish the diagnosis, treat symptoms,
assess those patients who may be at risk of sudden death and other complications and
advise on family screening.

Detailed family history and clinical examination

This can differentiate HCM from other conditions that can cause VH. The clinician
can establish whether there is a history of multiple premature sudden deaths or
recurrent syncope which can increase a patient’s ‘risk factor’ status. The carotid pulse is
rapid in upstroke, bifid and followed by a prominent dicrotic notch

12-lead ECG

This abnormal in 75-95% of patients with LVH and the abnormalities are not
specific to the disease. The ECG may be abnormal in family members who have
inherited the gene, but do not exhibit classic echocardiographic features. For example,
children and adolescents may demonstrate ECG abnormalities several years before they
display ventricular hypertrophy. The latter usually develops during periods of somatic
growth, such as in the first year of life or more usually during adolescence. Children
require regular investigations with ECG and to dimensional echo during growth spurts.
The most frequent change includes right and left atrial enlargement, repolarization
abnormalities and pathological Q - waves usually in the inferolateral leads.

Q- Waves are present in approximately 20-25% of patients. The exact cause of Q-

waves is not fully understood, but they may be due to myocardial ischemia, abnormal
septal activation and gross septal hypertrophy. As short P-R interval may present with a
slurred upstroke resembling wolff – Parkinson –White syndrome, but accessory
pathways are uncommon in patients with HCM. Left axis deviation is present in one
third of patients. Right axis deviation and right bundle branch block (RBBB) may be
present when the right ventricle is hypertrophied.

48-hour Holter monitoring

Around 20-25% of HCM patients have supraventricular arrhythmias which may be

paroxysmal or substained. Atrial fibrillation is the most common substained arrhythmia,
with paroxysmal episodes occurring in 20-25% of patients with HCM and is usually
linked to an enlarged left atrium. Most of these arrhythmias are slow in rate and the
patients are asymptomatic. They occur predominantly during periods of increased vagal
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tone, such as sleep. Some 25% of patients have episodes of non-substained ventricular
tachycardia (NSVT). The incidence of ventricular arrhythmias detected during 48-hour
monitoring is age-related and occurs mainly in adults. NSVT can be an increased risk
marker for sudden death. Substained VT is rare.

Echocardiography

This remains the gold standard for diagnosing HCM. It evaluates anatomical and
functional abnormalities underlining the presence of LVH and outflow obstruction. The
presence of a maximum wall thickness of 15 mm in the left ventricle, with no underlying
cause, is usually sufficient to make a diagnosis of HCM. Other characteristic
abnormalities include a small left ventricular cavity, hyper dynamic ventricle, SAM of the
mitral valve and valve abnormalities. Enlarged atria and impaired diastolic function can
also be seen. Doppler color flow can determine mitral regurgitation and can measure
pressure gradients. Echocardiography may not be as useful in children, but they should
undergo bi-annual echocardiograms during the adolescent growth spurt.

Exercise stress testing

This can be useful for obtaining functional and prognostic information. Patients with
HCM have an impaired maximal oxygen ventilator capacity, which may be due to an
inability to increase stroke volume during exercise. About 20-25% of patients have an
abnormal blood pressure response to exercise; this is defined as a failure of the systolic
blood pressure to rise by over 20 mm Hg during exercise. Alternatively, it may fall from
the resting baseline measurement. The mechanism for this abnormal response is
unclear, but it may be due to ventricular baroreceptors causing inappropriate
vasodilatation. Patients under the age of 40 years who present with flat blood pressure
or hypotensive responses, have a higher risk of sudden death, however as a single risk
factor, its positive predictive value is low.

Chest X-ray

This may be normal or revel cardiomegaly due to bi-atrial enlargement and left
ventricular hypertrophy. Occasional mitral annular calcification can be seen, while upper
lobe blood diversion may be identified in patients with long-standing raised left atrial
pressures.
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Cardiac catheterization

This is usually reserved for patients who require pressure measurements for severe
clinical mitral regurgitation. Coronary angiography is indicated in patients with chest
pain above 40 years of age, to exclude underlying CHD.

MRI

This can be useful in assessing right ventricular and apical hypertrophy in selected
patients.
MANAGEMENT
Medical management

The main goals of management of patients with HCM are to decrease the risk of sudden
cardiac death and treat symptoms of dyspnea, angina, fatigue, and syncope. An
automatic implantable cardioverter-defibrillator has been used with success after
sudden death was the presenting symptom. Thus far, cardiac rhythm disturbances are
believed to be the most common cause of sudden cardiac death in HCM. Non sustained
ventricular tachycardia has been frequently found on 24-hour Holter monitoring.
Ventricular fibrillation and ventricular tachycardia have also been implicated, especially
when patients have a history of syncope, pre syncope, or prior cardiac arrest.

Medical management includes the use of beta blockers, calcium channel blockers, and
anti arrhythmics.

Beta blockers, such as propranolol hydrochloride, decrease outflow tract obstruction

and the heart rate, allowing greater time for filling of the chambers. Oxygen demand is
decreased, therefore decreasing angina. The rationale for beta blocker use is inhibition
of sympathetic stimulation of the heart, resulting in diminished left ventricular
contractility and reduced myocardial oxygen demand at rest and during exercise. Beta
blockers can relieve most symptoms of HCM, including angina, dyspnea, pre syncope, or
syncope. However, beta blockers have not produced sustained effects on HCM in many
patients, and frequently deterioration results.

Calcium channel blockers improve the rate of relaxation and decrease heart rate and
blood pressure. Calcium channel blockers, such as verapamil, reduce LVOTO. A decrease
in systolic function and an improvement in diastolic relaxation and filling results from
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calcium channel blocker use. However, long-term success has also been a problem with
these agents.

Quinidine and procainamide hydrochloride have been used to suppress ventricular

arrhythmias. Disopyramide, a type I antiarrhythmic with negative inotropic properties,
has been advocated for patients with HCM and LVOTO. Varying results with its use have
been reported, but long-term results, as with the other therapies, have shown symptom
improvement for some patients.

Amiodarone is a potent class III antiarrhythmic agent that shows slow calcium channel,
fast sodium channel, and b-adrenergic blocking properties. It has been shown to lessen
symptoms caused by LVOTO, to increase exercise tolerance, and to improve left
ventricular compliance and diastolic filling in patients with HCM.

SURGICAL THERAPY

Subaortic ventricular myotomy was first performed on two patients in 1961. Now the
popularity of treatment was varied. Here myocardium from the proximal septum just
beyond the mitral leaflets is resected to reduce the outflow gradient. Advantages are
low mortality rate, reduced symptoms, and improved functional capacity, symptomatic
improvement persists for 5 or more years after surgery in 70% of patients.
 Septal myectomy. This is an open-heart operation in which the surgeon removes
part of the thickened, overgrown heart muscle wall (septum) that separates the two
bottom heart chambers (ventricles). Removing part of this overgrown muscle improves
blood flow and reduces mitral regurgitation. Myectomy is used if medications don't
relieve symptoms. Most people who have symptoms and undergo myectomy have no
further symptoms. This type of surgery is available only in medical centers that
specialize in the treatment of hypertrophic cardiomyopathy.
 Septal ablation. Also called septal alcohol ablation, this is a treatment in which a
small portion of the thickened heart muscle is destroyed by injecting alcohol through a
catheter into the artery supplying blood to it. There are possible complications with this
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procedure, including heart block — a disruption of the heart's electrical system — which
requires implantation of a pacemaker.
 Pacemaker implantation. A pacemaker is a small electronic device inserted
under your skin that sends electrical signals to your heart to monitor and regulate your
heartbeat. Surgery to implant the pacemaker is usually performed under local
anesthesia and typically takes less than three hours. Pacemaker implantation is
generally not as effective as surgical options, but it's sometimes used in older people
who want to avoid more invasive procedures.
 Implantable cardioverter-defibrillator (ICD). This is a pager-sized device
implanted in your chest like a pacemaker. An ICD continuously monitors your heartbeat.
If a life-threatening arrhythmia occurs, the ICD delivers precisely calibrated electrical
shocks to restore a normal heart rhythm. Some people with hypertrophic
cardiomyopathy are at risk of sudden cardiac death because of abnormal heart rhythms.
In these high-risk individuals, many doctors recommend the implantation of an ICD.

Heart transplantation

It is an option for end stage HCM patients with deterioration in LV systolic function who
exhibit debilitating symptoms and whom heart failure develops. The first human-to-
human heart transplant was performed in 1967. Since then, transplant procedures,
equipment, and medications have continued to improve. Since1983, when cyclosporine
became available, heart transplantation has become a therapeutic option for patients
with end-stage heartdisease. Cyclosporine (Neoral, Sandimmune, SangCya) is an
immunosuppressant that greatly decreases the body’s rejection of foreign proteins,
such as transplanted organs. Unfortunately, cyclosporine also decreases the body’s
ability to resist infections, and a satisfactory balance must be achieved between
suppressing rejection and avoiding infection. Cardiomyopathy, ischemic heart disease,
valvular disease, rejection of previously transplanted hearts, and congenital heart
disease are the most common indications for transplantation

A typical candidate has severe symptoms uncontrolled by medical therapy, no other

surgical options, and a prognosis of less than 12 months to live. A multidisciplinary team
 19

screens the candidate before recommending the transplantation procedure. The

person’s age, pulmonary status, other chronic health conditions, psychosocial status,
family support, infections, history of other transplantations, compliance, and current
health status are considered in the screening. When a donor heart becomes available, a
computer generates a list of potential recipients on the basis of ABO blood group
compatibility, the sizes of the donor and the potential recipient, and the geographic
locations of the donor and potential recipient; distance is a variable because
postoperative function depends on the heart being implanted within 6 hours of harvest
from the donor. Some patients are candidates for more than one organ transplant:
heart-lung, heart-pancreas, heart-kidney, heart-liver.

Transplantation Techniques
Orthotopic transplantation is the most common surgical procedure for cardiac
transplantation. The recipient’s heart is removed, and the donor heart is implanted at
the vena cava and pulmonary veins. Some surgeons still prefer to remove the recipient’s
heart leaving a portion of the recipient’s atria (with the vena cava and pulmonary veins)
in place. The donor heart, which usually has been preserved in ice, is prepared for
implant by cutting away a small section of the atria that corresponds with the sections
of the recipient’s heart that were left in place. The donor heart is implanted by suturing
the donor atria to the residual atrial tissue of the recipient’s heart. Both techniques then
connect the recipient’s pulmonary artery and aorta to those of the donor heart.
Heterotopic transplantation is less commonly performed the donor heart is placed to
the right and slightly anterior to the recipient’s heart; the recipient’s heart is not
removed. Initially, it was thought that the original heart might provide some protection
for the patient in the event that the transplanted heart was rejected. Although the
protective effect has not been proved, other reasons for retaining the original heart
have been identified: a small donor heart or pulmonary hypertension. The transplanted
heart has no nerve connections with the recipient’s body (i.e., denervated heart), and
the sympathetic and vagus nerves do not affect the transplanted heart. The resting rate
of the transplanted heart is approximately 70 to 90 beats per minute, but it increases
gradually if catecholamines are in the circulation. Patients must gradually increase and
decrease their exercise (i.e., extended warm-up and cool-down periods), because 20 to
30 minutes may be required to achieve the desired heart rate. Atropine does not
increase the heart rate of these patients.
Postoperative Course
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Heart transplant patients are constantly balancing the risk of rejection with the
risk of infection. They must comply with a complex regimen of diet, medications,
activity, follow-up laboratory studies, biopsies (to diagnose rejection), and clinic visits.
Most commonly, patients receive cyclosporine or tacrolimus (Prograf), azathioprine
(Imuran) or mycophenolate mofetil (CellCept), and corticosteroids (ie, prednisone) to
minimize rejection. In addition to rejection and infection, complications may include
accelerated atherosclerosis of the coronary arteries (i.e., cardiac allograft vasculopathy
[CAV] or accelerated graft atherosclerosis [AGA]). Although the cause is unknown, the
disease is believed to be immunologically mediated. Hypertension may be experienced
by patients taking cyclosporine or tacrolimus; the cause has not been identified.
Osteoporosis frequently occurs as a side effect of the anti-rejection medications
and pre transplantation dietary insufficiency and medications. Post transplantation
lympho proliferative disease and cancer of the skin and lips are the most common
malignancies after transplantation, possibly caused by immunosuppression. Weight
gain, obesity, diabetes, dyslipidemias (e.g., hypercholesterolemia), hypotension, renal
failure, and central nervous system, respiratory, and gastrointestinal disturbances may
be caused by the corticosteroids or other immunosuppressants. Other complications are
immunosuppressant medication toxicities and responses to the psychosocial stresses
imposed by organ transplantation. Patients may experience guilt that someone died for
them to live, have anxiety about the new heart, experience depression or fear when
rejection is identified, or have difficulty with family role changes before and after
transplantation. The 1-year survival rate for patients with transplanted hearts is
approximately 80% to 90%; the 5-year survival rate is approximately 60% to 70%.
Mechanical Assist Devices and Total Artificial Hearts
The use of cardiopulmonary bypass for cardiovascular surgery and the possibility
of performing heart transplantation for end-stage cardiac disease have increased the
need for mechanical assist devices. Patients who cannot be weaned from
cardiopulmonary bypass or patients in cardiogenic shock may benefit from a period of
mechanical heart assistance. The most commonly used device is the intra-aortic balloon
pump. This pump decreases the work of the heart during contraction but does not
perform the actual work of the heart.
Ventricular Assist Devices
More complex devices that actually perform some or all of the pumping function
for the heart also are being used. These more sophisticated ventricular assist devices
(VADs) can circulate as much blood per minute as the patient’s heart, if not more. Each
 21

ventricular assist device is used to support one ventricle. Some ventricular assist devices
can be combined with an oxygenator; the combination is called extracorporeal
membrane oxygenation (ECMO). The oxygenator– ventricular assist device combination
is used for the patient whose heart cannot pump adequate blood through the lungs or
the body.
There are three basic types of devices: centrifugal, pneumatic, and electric or
electromagnetic. Centrifugal VADs are external, non-pulsatile, cone-shaped devices with
internal mechanisms that spin rapidly, creating a vortex (tornado-like action) that pulls
blood from a large vein into the pump and then pushes it back into a large artery.
Pneumatic VADs are external or implanted pulsatile devices with a flexible reservoir
housed in a rigid exterior. The reservoir usually fills with blood drained from the
patient’s atrium or ventricle. The VAD then forces pressurized air into the rigid housing,
compressing the reservoir and returning the blood to the patient’s circulation, usually
into the aorta. Electric or electromagnetic VADs are similar to the pneumatic VADs, but
instead of pressurized air, one or more flat metal plates are pushed against the reservoir
to return the blood to the patient’s circulation.
Total Artificial Hearts
Total artificial hearts are designed to replace both ventricles. Some require the
removal of the patient’s heart to implant the total artificial heart; others do not. All of
these devices are experimental. Although there has been some short-term success, the
long-term results have been disappointing. Researchers hope to develop a device that
can be permanently implanted and that will eliminate the need for donated human
heart transplantation for the treatment of end-stage cardiac disease. Most VADs and
total artificial hearts are temporary treatments while the patient’s own heart recovers
or until a donor heart becomes available for transplantation (ie, “bridge to transplant”).
Some devices are being investigated for permanent use. Bleeding disorders,
hemorrhage, thrombus, emboli, hemolysis, infection, renal failure, right heart failure,
multisystem failure, and mechanical failure are some of the complications of VADs and
total artificial hearts. The nursing care for these patients focuses on assessing for and
minimizing these complications and involves providing emotional support and education
about the mechanical assist device.

Other treatment considerations

Alcohol ablation of the septum

 22

Here approximately 1 to 4 mL of absolute alcohol is injected selectively into the septal

perforator branch of the left anterior descending artery through a percutaneous
catheter. The resultant MI reduces the thickness of the proximal septum. Thus the
outflow tract dimension is increased and the gradient is reduced.

Permanent pacing

Now permanent pacing in HCM patients is reduced over recent years because of
decrease in cardiac output and stroke volume. So now it is considered as an alternative
procedure elderly subgroup.

RESTRICTIVE CARDIOMYOPATHY

DEFINITION

It is a group of disorders. The heart chambers are unable to fill with blood because the
heart muscle is stiff. Restrictive cardiomyopathy is characterized by a primary
abnormality of diastolic ventricular function with normal to near-normal systolic
function and normal ventricular internal dimensions.

PREVALENCE
Restrictive cardiomyopathy is the least common type of cardiomyopathy seen in
Western countries. It is more common in Africa than in other parts of the world.
ETIOLOGY AND RISK FACTORS

Although the specific etiology of restrictive CMP is unknown, a number of pathologic

processes may be involved in its development. It is possible to divide the causes into
primary and secondary.

Primary

 Löffler's endocarditis
 endocardial fibroelastosis

Secondary

 infiltrative
 23

o cardiac amyloidosis
o haemochromatosis
o sarcoidosis

 interstitial
o postradiation fibrosis

Other causes include scleroderma, Churg-Strauss syndrome, cystinosis, lymphoma,

Gaucher's disease, hemochromatosis, Fabry's disease, pseudoxanthoma elasticum,
hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, and
Werner's syndrome

Other Restrictive Cardiomyopathies

Iron-overload cardiomyopathy (hemochromatosis) is often the result of multiple

transfusions or a hemoglobinopathy, most frequently -thalassemia; the familial
(autosomal recessive) form should be suspected if cardiomyopathy occurs in the
presence of diabetes mellitus, hepatic cirrhosis, and increased skin pigmentation. The
diagnosis may be confirmed by endomyocardial biopsy. CMRI shows a reduced T2*
signal as iron levels rise. Phlebotomy may be of some benefit if employed early in the
course of the disease. Continuous subcutaneous administration of deferoxamine or
other iron chelators may reduce body iron stores and result in clinical improvement.

Myocardial sarcoidosis is generally associated with other manifestations of systemic

disease. It may cause restrictive as well as congestive features, since cardiac infiltration
by sarcoid granulomas results not only in increased stiffness of the myocardium but also
in diminished systolic contractile function. A variety of arrhythmias, including high-grade
AV block, have been noted. A common cardiac manifestation of systemic Sarcoidosis is
RV overload due to pulmonary hypertension as a result of parenchymal pulmonary
involvement. Many patients are treated empirically with glucocorticoids.

The carcinoid syndrome results in endocardial fibrosis and stenosis and/or regurgitation
of the tricuspid and/or pulmonary valve; morphologically similar lesions have been seen
with the use of the anorexic agent’s fenfluramine and phentermine.

PATHOPHYSIOLOGY
 24

Fibrotic infiltrations into the myocardium, endocardium and sub endocardium

cause the ventricles to lose their ability to stretch. The excessively rigid ventricular walls
impair filling during diastole; however, contractility with systole is usually normal. Filling
pressure increase and cardiac output falls. Eventually cardiac failure and mild ventricular
hypertrophy occurs.

Clinical Manifestations

Classic symptoms of restrictive CMP are fatigue, exercise intolerance, and

dyspnea because the heart cannot increase CO by increasing the heart rate without
further compromising ventricular filling. Additional symptoms may include angina,
orthopnea, syncope, and palpitations. The patient may have signs of HF, including
dyspnea, peripheral edema, ascites, hepatomegaly, and jugular venous distention.

Diagnostic Studies

Laboratory tests shows hemochromatosis, that is, increased serum iron, ferritin.

The chest x-ray may be normal, or it may show cardiomegaly from right and left atrial
enlargement. Pleural effusions and pulmonary congestion may be evident in the patient
with progression to HF.

The ECG may reveal a mild tachycardia at rest. The most common dysrhythmias are
supraventricular (atrial fibrillation) or atrioventricular block.

Echocardiography may reveal a left ventricle that is normal sized with a thickened wall,
slightly dilated right ventricle, and dilated atria.

CT scan, MRI and nuclear imaging may be helpful in the diagnosis. This can provide
ventricular volumetric changes of each heartbeat. Pericardial thickening (2mm or more)
can be detected in CT and MRI.

Cardiac catheterization shows rapid early diastolic filling in LV and RV, with dip and
plateau waveform.

Endomyocardial biopsy is often limited value in DCM, but it may helpful here. In cardiac
amyloidosis, histochemical staining helps to distinguish primary AL type
(immunoglobulin chains).
 25

MANAGEMENT

The treatment of diastolic heart failure centers on reducing symptoms. When diastolic
pressures are elevated, diuretics can be used. But the overuse of diuretics can result in
hypotension, renal dysfunction. Increased bowel edema reduces the absorption of
frusemide. Oral torsemide is a preferred diuretic when bowel edema is present.

Spironolactone is a useful adjunct, especially if liver congestion and ascites are preset.

Beta blockers can improve rate control.

Calcium channel blockers are also used routinely that they can improve e myocardial
diastolic dysfunction in addition to helping control ventricular rate in patients with atrial
fibrillation, thereby improving cardiac function.

ACE inhibitors may also improve myocardial relaxation and are often useful despite
relatively normal ventricular systolic function.

Digoxin may result in increased arrhythmias, especially in patients with amyloidosis

which is not used

SURGICAL MANAGEMENT

When medical therapy fails, cardiac transplantation is the choice. But amyloidosis has
been reported in transplanted heart so that it is not appropriate for patients with
systemic amyloidosis

SPECIFIC THERAPY

Liver transplantation may be an option in familial amyloidosis, because the circulating

transthyretin that causes the disorder is manufactured in the liver.

Corticosteroids and hydrooxyurea are used in early stages of hypereosinophilic

syndromes. There has also been some success with interferon in this disease. Surgery
can debride the fibrous plaque, and valve replacement may be indicated.

Enzyme replacement and liver transplantation has improved in some patients with
Gaucher’s disease.
 26

Hemochromatosis is managed by phlebotomy, chelating agents such as desferrioxamine

or both. Herat transplantation and combined heart-liver transplantation have also been
used in patients whose hemochromatosis is refractory to standard therapy.

Fabry’s disease can now be treated with intermittent intravenous infusion of the
enzyme alpha-galactosidase A.

DIALATED RESTRICTIVE HYPERTROPHIC

Pathophysiolo Fibrosis of myocardium Hypertrophy of all walls Mimic constrictive

gy and endocardium Hypertrophied septum pericarditis
Dilated chambers Relatively small chamber Fibrosed walls cannot
Mural wall thrombi size expand or contract
prevalent Chambers narrowed:
emboli common
Symptoms Congestive heart failure, Dyspnea, fatigue, Right- Dyspnea, angina
particularly left sided, sided congestive heart pectoris, Fatigue.,
fatigue or weakness, failure, angina, Signs and syncope, palpitations
systemic or pulmonary symptoms of systemic
emboli disease, e.g.,
amyloidosis, iron storage
disease
Physical Moderate to severe Mild to moderate Mild cardiomegaly,
examination cardiomegaly; S3, S4 cardiomegaly; S3 or S4 Apical systolic thrill and
Atrioventricular valve Atrioventricular valve heave; brisk carotid
regurgitation, especially regurgitation; inspiratory upstroke, S4 common,
mitral increase in venous Systolic murmur that
pressure (Kussmaul sign) increases with Valsalva
maneuver
Chest Moderate to marked Mild cardiac silhouette Mild to moderate
roentgenogra cardiac silhouette enlargement cardiac silhouette
m enlargement enlargement

Pulmonary venous
hypertension
Electrocardiog ST-segment and T-wave Low voltage, conduction ST-segment and T-
ram abnormalities defects wave abnormalities
Left ventricular
 27

hypertrophy
Abnormal Q waves
Echocardiogra Left ventricular Increased left ventricular Asymmetric septal
m dilatation and wall thickness hypertrophy (ASH)
dysfunction Normal or mildly Systolic anterior
reduced systolic function motion (SAM) of the
mitral valve
Radionuclide Left ventricular Normal or mildly Vigorous systolic
studies dilatation and reduced systolic function function (RVG)
dysfunction (RVG) Perfusion defect
Cardiac Left ventricular Normal or mildly Vigorous systolic
catheterizatio dilatation and reduced systolic function function
n dysfunction Elevated left- and right- Dynamic left
sided filling pressures ventricular outflow
Elevated left- and often obstruction
right-sided filling Elevated left- and
pressures right-sided filling
pressures
Diminished cardiac
output
Treatment Symptomatic treatment Symptomatic treatment Supportive treatment
of heart failure Beta blockers of symptoms
Conversion of atrial Treatment of
Vasodilators fibrillation hypertension
Ventriculomyotomy or Exercise restrictions
Control of dysrhythmias muscle resection with Emergency treatment
mitral valve replacement of acute pulmonary
Heart transplantation edema

ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

ARVC occurs when the myocardium of the right ventricle is progressively infiltrated and
replaced by fibrous scar and adipose tissue. Initially, only localized areas of the right
ventricle are affected, but as the disease progresses, the entire heart is affected.

Incidence
 28

The incidence of ARVD is about 1/10,000 in the general population in the United States,
although some studies have suggested that it may be as common as 1/1,000. Recently,
1/200 was found to be carriers of mutations that predispose to ARVC. It accounts for up
to 17% of all sudden cardiac deaths in the young. In Italy, the incidence is 40/10,000,
making it the most common cause of sudden cardiac death in the young population.

Pathogenesis

The pathogenesis of ARVD is largely unknown. Apoptosis (programmed cell death)

appears to play a large role. It is unclear why only the right ventricle is involved. The
disease process starts in the subepicardial region and works its way towards the
endocardial surface, leading to transmural involvement (possibly accounting for the
aneurysmal dilatation of the RV). Residual myocardium is confined to the
subendocardial region and the trabeculae of the RV. These trabeculae may become
hypertrophied.

There are two pathological patterns seen in ARVD, Fatty infiltration and fibro-fatty
infiltration.

Fatty infiltration

The first, fatty infiltration is confined to the right ventricle. This involves a partial or
near-complete substitution of myocardium with fatty tissue without wall thinning. It
involves predominantly the apical and infundibular regions of the RV. The left ventricle
and ventricular septum are usually spared. No inflammatory infiltrates are seen in fatty
infiltration. There is evidence of myocyte (myocardial cell) degeneration and death seen
in 50% of cases of fatty infiltration.

Fibro-fatty infiltration

The second, fibro-fatty infiltration, involves replacement of myocytes with fibrofatty

tissue. A patchy myocarditis is involved in up to 2/3 of cases, with inflammatory
infiltrates (mostly T cells) seen on microscopy. Myocardial atrophy is due to injury and
apoptosis. This leads to thinning of the RV free wall (to < 3 mm thickness) Myocytes are
replaced with fibrofatty tissue. The regions preferentially involved include the RV inflow
tract, the RV outflow tract, and the RV apex. However, the LV free wall may be involved
 29

in some cases. Involvement of the ventricular septum is rare. The areas involved are
prone to aneurysm formation.

Ventricular arrhythmias

Ventricular arrhythmias due to ARVD typically arise from the diseased right ventricle.
The type of arrhythmia ranges from frequent premature ventricular complexes (PVCs) to
ventricular tachycardia (VT) to ventricular fibrillation (VF).

While the initiating factor of the ventricular arrhythmias is unclear, it may be due to
triggered activity or reentry.

Ventricular arrhythmias are usually exercise-related, suggesting that they are sensitive
to catecholamines. The ventricular beats typically have a right axis deviation. Multiple
morphologies of ventricular tachycardia may be present in the same individual,
suggesting multiple arrhythmogenic foci or pathways.

Right ventricular outflow tract (RVOT) tachycardia is the most common VT seen in
individuals with ARVD. In this case, the EKG shows a left bundle branch block (LBBB)
morphology with an inferior axis.

CLINICAL PRESENTATION

Up to 80% of individuals with ARVD present with syncope or sudden cardiac death. The
remainder frequently presents with palpitations or other symptoms due to right
ventricular outflow tract (RVOT) tachycardia (a type of monomorphic ventricular
tachycardia).

Symptoms are usually exercise-related. In populations where hypertrophic

cardiomyopathy is screened out prior to involvement in competitive athletics, it is a
common cause of sudden cardiac death.

The first clinical signs of ARVD are usually during adolescence. However, signs of ARVD
have been demonstrated in infants.
 30

DIAGNOSIS

In order to make the diagnosis of ARVD, a number of clinical tests are employed,
including the electrocardiogram (EKG), echocardiography, right ventricular angiography,
cardiac MRI, and genetic testing.

Electrocardiogram

90% of individuals with ARVD have some EKG abnormality. The most common EKG
abnormality seen in ARVD is T wave inversion in leads V 1 to V3. However, this is a non-
specific finding, and may be considered a normal variant in right bundle branch block
(RBBB), women, and children under 12 years old.

RBBB itself is seen frequently in individuals with ARVD. This may be due to delayed
activation of the right ventricle, rather than any intrinsic abnormality in the right bundle
branch.

The epsilon wave is found in about 50% of those with ARVD. This is described as a
terminal notch in the QRS complex. It is due to slowed intraventricular conduction.
Echocardiography

Echocardiography may reveal an enlarged, hypokinetic right ventricle with a paper-thin

RV free wall. The dilatation of the RV will cause dilatation of the tricuspid valve annulus,
with subsequent tricuspid regurgitation. Paradoxical septal motion may also be present.

Cardiac MRI
MRI in a patient affected by ARVC/D (long axis view of the right ventricle): note the
transmural diffuse bright signal in the RV free wall on spin echo T1 (a) due to massive
myocardial atrophy with fatty replacement

Fatty infiltration of the RV free wall can be visible on cardiac MRI. Fat has increased
intensity in T1-weighted images. However, it may be difficult to differentiate
intramyocardial fat and the epicardial fat that is commonly seen adjacent to the normal
heart. Also, the sub-tricuspid region may be difficult to distinguish from the
atrioventricular sulcus, which is rich in fat.
 31

Cardiac MRI can visualize the extreme thinning and akinesis of the RV free wall.
However, the normal RV free wall may be about 3 mm thick, making the test less
sensitive.

Right ventricular angiography

Right ventricular angiography is considered the gold standard for the diagnosis of ARVD.
Findings consistent with ARVD are an akinetic or dyskinetic bulging localized to the
infundibular, apical, and subtricuspid regions of the RV. The specificity is 90%; however,
the test is observer dependent.

Right ventricular biopsy

Transvenous biopsy of the right ventricle can be highly specific for ARVD, but it has low
sensitivity. False positives include other conditions with fatty infiltration of the ventricle,
such as chronic alcohol abuse and Duchenne/Becker muscular dystrophy.

False negatives are common, however, because the disease progresses typically from
the epicardium to the endocardium (with the biopsy sample coming from the
endocardium), and the segmental nature of the disease. Also, due to the paper-thin
right ventricular free wall that is common in this disease process, most biopsy samples
are taken from the ventricular septum, which is commonly not involved in the disease
process.

A biopsy sample that is consistent with ARVD would have > 3% fat, >40% fibrous tissue,
and <45% myocytes.

Autopsy
In vitro MRI and corresponding cross section of the heart in ARVD show RV dilatation
with anterior and posterior aneurysms (17 year old asymptomatic male athlete who
died suddenly during soccer game).

A post mortem histological demonstration of full thickness substitution of the RV

myocardium by fatty or fibro-fatty tissue is consistent with ARVD.
 32

Genetic Testing

ARVD is an autosomal dominant trait with reduced penetrance. Approximately 40-50%

of ARVD patients have a mutation identified in one of several genes encoding
components of the desmosome, which can help confirm a diagnosis of ARVD. [6] Since
ARVD is an autosomal dominant trait, children of an ARVD patient have a 50% chance of
inheriting the disease causing mutation. Whenever a mutation is identified by genetic
testing, family-specific genetic testing can be used to differentiate between relatives
who are at-risk for the disease and those who are not. ARVD genetic testing is clinically
available.

Diagnostic Criteria

There is no pathognomonic feature of ARVD. The diagnosis of ARVD is based on a

combination of major and minor criteria. To make a diagnosis of ARVD requires either 2
major criteria or 1 major and 2 minor criteria or 4 minor criteria.

Major Criteria

 Right ventricular dysfunction

o Severe dilatation and reduction of RV ejection fraction with little or no LV
impairment
o Localized RV aneurysms
o Severe segmental dilatation of the RV
 Tissue characterization
o Fibrofatty replacement of myocardium on endomyocardial biopsy
 Conduction abnormalities
o Epsilon waves in V1 - V3.
o Localized prolongation (>110 ms) of QRS in V1 - V3
 Family history
o Familial disease confirmed on autopsy or surgery

Minor Criteria

 Right ventricular dysfunction

o Mild global RV dilatation and/or reduced ejection fraction with normal LV.
 33

o Mild segmental dilatation of the RV

o Regional RV hypokinesis
 Tissue characterization
 Conduction abnormalities
o Inverted T waves in V 2 and V3 in an individual over 12 years old, in the
absence of a right bundle branch block (RBBB)
o Late potentials on signal averaged EKG.
o Ventricular tachycardia with a left bundle branch block (LBBB) morphology
o Frequent PVCs (> 1000 PVCs / 24 hours)
 Family history
o Family history of sudden cardiac death before age 35
o Family history of ARVD

MANAGEMENT

The goal of management of ARVD is to decrease the incidence of sudden cardiac death.
This raises a clinical dilemma: How to prophylactically treat the asymptomatic patient
who was diagnosed during family screening.

A certain subgroup of individuals with ARVD is considered at high risk for sudden cardiac
death. Characteristics associated with high risk of sudden cardiac death include:

 Young age
 Competitive sports activity
 Malignant familial history
 Extensive RV disease with decreased right ventricular ejection fraction.
 Left ventricular involvement
 Syncope
 Episode of ventricular arrhythmia

Management options include pharmacological, surgical, catheter ablation, and

placement of an implantable cardioverter-defibrillator.

Prior to the decision of the treatment option, programmed electrical stimulation in the
electrophysiology laboratory may be performed for additional prognostic information.
Goals of programmed stimulation include:
 34

 Assessment of the disease's arrhythmogenic potential

 Evaluate the hemodynamic consequences of sustained VT
 Determine whether the VT can be interrupted via antitachycardia pacing.

Regardless of the management option chosen, the individual is typically suggested to

undergo lifestyle modification, including avoidance of strenuous exercise, cardiac
stimulants (i.e.: caffeine, nicotine, pseudoephedrine) and alcohol. If the individual
wishes to begin an exercise regimen, an exercise stress test may have added benefit.

Pharmacologic management

Pharmacologic management of ARVD involves arrhythmia suppression and prevention

of thrombus formation.

Sotalol, a beta blocker and a class III antiarrhythmic agent, is the most effective
antiarrhythmic agent in ARVD. Other antiarrhythmic agents used include amiodarone
and conventional beta blockers (i.e.: metoprolol). If antiarrhythmic agents are used,
their efficacy should be guided by series ambulatory holter monitoring, to show a
reduction in arrhythmic events.

While angiotensin converting enzyme inhibitors (ACE Inhibitors) are well known for
slowing progression in other cardiomyopathies, they have not been proven to be helpful
in ARVD.

Individuals with decreased RV ejection fraction with dyskinetic portions of the right
ventricle may benefit from long term anticoagulation with warfarin to prevent thrombus
formation and subsequent pulmonary embolism.

Catheter ablation

Catheter ablation may be used to treat intractable ventricular tachycardia. It has a 60-
90% success rate unfortunately, due to the progressive nature of the disease,
recurrence is common (60% recurrence rate), with the creation of new arrhythmogenic
foci. Indications for catheter ablation include drug-refractory VT and frequent
recurrence of VT after ICD placement, causing frequent discharges of the ICD.
 35

Implantable cardioverter-defibrillator

An ICD is the most effective prevention against sudden cardiac death. Due to the
prohibitive cost of ICDs, they are not routinely placed in all individuals with ARVD.

Indications for ICD placement in the setting of ARVD include:

 Cardiac arrest due to VT or VF

 Symptomatic VT that is not inducible during programmed stimulation
 Failed programmed stimulation-guided drug therapy
 Severe RV involvement with poor tolerance of VT
 Sudden death of immediate family member

Since ICDs are typically placed via a transvenous approach into the right ventricle, there
are complications associated with ICD placement and follow-up.

Due to the extreme thinning of the RV free wall, it is possible to perforate the RV during
implantation, potentially causing pericardial tamponade. Because of this, every attempt
is made at placing the defibrillator lead on the ventricular septum.

After a successful implantation, the progressive nature of the disease may lead to fibro-
fatty replacement of the myocardium at the site of lead placement. This may lead to
under sensing of the individual's electrical activity (potentially causing inability to sense
VT or VF), and inability to pace the ventricle.

Cardiac transplant surgery

Cardiac transplant surgery may be performed in ARVD. It may be indicated if the

arrhythmias associated with the disease are uncontrollable or if there is severe bi-
ventricular heart failure that is not manageable with pharmacological therapy.

Family screening

All first degree family members of the affected individual should be screened for ARVD.
This is used to establish the pattern of inheritance. Screening should begin during the
teenage years unless otherwise indicated. Screening tests include:

 Echocardiogram
 36

 EKG
 Signal averaged EKG
 Holter monitoring
 Cardiac MRI
 Exercise stress test

UNCLASSIFIED CARDIOMYOPATHIES
Unclassified cardiomyopathies are different from or have characteristics of more than
one of the previously described cardiomyopathies. Examples of unclassified
cardiomyopathies include fibroelastosis, noncompacted myocardium, systolic
dysfunction with minimal dilation, and mitochondrial involvement.

Left Ventricular Non-compaction

Left ventricular non-compaction (LVNC) is a recently characterized uncommon

congenital cardiomyopathy that may present at any age with symptoms of CHF,
thromboembolism, or ventricular arrhythmias. It results from the arrest of normal
embryogenesis, with the persistence of the deep recesses and sinusoids in the
myocardium that characterize the embryonic heart. These sinusoids and associated
spongy network of myocardial fibers ordinarily undergo organization and "compaction"
early in embryonic life; when this fails to occur, LVNC results. This condition is diagnosed
on echocardiography by the demonstration of multiple deep trabeculations into the
myocardium, all of which communicate with the ventricular cavity, associated with LV
contractile dysfunction. Standard therapy for CHF is routinely employed, typically along
with chronic anticoagulation.

COMPLICATIONS

Heart failure

It means the heart can’t pump enough blood to meet the body’s needs. The weakened,
thickened and stiffened heart muscle due to cardiomyopathy become unable to pump
or can stop blood from flowing out of the heart.

Blood clots
 37

As heart can’t pump effectively, there is a chance of blood clots due to cardiomyopathy.
If clots are pumped out of heart will enter the blood stream, they block the blood flow
to other organs, including heart and brain. If clots develop on right side of heart, they
may travel to lungs.

Valve problems

Because people with dilated cardiomyopathy have an enlarged heart, two of the heart
valves- the mitral and tricuspid valves may not close properly, lead to backward flow of
blood. This flow creates murmurs.

Cardiac arrest and sudden death

All forms of cardiomyopathy can lead to abnormal heart rhythms. Some of these heart
rhythms are too slow to keep blood flowing through the heart effectively, and some are
too fast to allow the heart to beat properly.in either case, these abnormal rhythms can
result in fainting or sudden death if heart stops beating.

NURSING MANAGEMENT
Assessment
Nursing assessment for the patient with cardiomyopathy begins with a detailed
history of the presenting signs and symptoms. The nurse identifies possible etiologic
factors, such as heavy alcohol intake, recent illness or pregnancy, or history of the
disease in immediate family members. If the patient complains of chest pain, a thorough
review of the pain, including its precipitating factors, should be performed. The review
of systems includes the presence of orthopnea, paroxysmal nocturnal dyspnea, and
syncope or dyspnea with exertion. The number of pillows that are needed to sleep,
usual weight, any weight change, and limitation to activities of daily living also are
assessed. The New York Heart Association Classification for heart failure is determined.
The patient’s usual diet is evaluated to determine if alterations are needed to reduce
sodium intake.
Because of the chronicity of cardiomyopathy, the nurse compiles a careful
psychosocial history exploring the impact of the disease on the patient’s role within the
family and community. Identification of all perceived stressors helps the patient and the
health care team to implement activities to relieve anxiety related to changes in health
status. Very early on, the patient’s support systems are identified, and members are
 38

involved in the patient’s care and therapeutic regimen. The assessment addresses the
effect the diagnosis has had on the patient and members of his or her support system
and the patient’s emotional status. Depression is not uncommon in patients with
cardiomyopathy who have developed heart failure. The physical assessment focuses on
signs and symptoms of congestive heart failure. The baseline assessment includes such
key components as:
• Vital signs
• Calculation of pulse pressure and identification of pulsus paradoxus
• Current weight; determination of weight gain or loss
• Detection by palpation of the point of maximal impulse, often shifted to the left
• Cardiac auscultation for a systolic murmur and third and fourth heart sounds
• Pulmonary auscultation for crackles
• Measurement of jugular vein distention
• Identification of presence and severity of edema
DIAGNOSIS
 Decreased cardiac output related to structural disorders caused by
cardiomyopathy or to dysrhythmia from the disease process and medical
treatments
 Ineffective cardiopulmonary, cerebral, peripheral, and renal tissue perfusion
related to decreased peripheral blood flow (resulting from decreased cardiac
output)
 Impaired gas exchange related to pulmonary congestion caused by myocardial
failure (decreased cardiac output)
 Activity intolerance related to decreased cardiac output or excessive fluid
volume, or both
 Anxiety related to the change in health status and in role functioning
 Powerlessness related to disease process
 Noncompliance with medication and diet therapies
Planning and Goals
The major goals for the patient include improved or maintained cardiac output,
increased activity tolerance, reduction of anxiety, adherence to the self-care program,
increased sense of power with decision making, and absence of complications.
NURSING INTERVENTIONS
 IMPROVING CARDIAC OUTPUT
 39

During a symptomatic episode, rest is indicated. Many patients with DCM find that
sitting up with their legs down is more comfortable than lying down in a bed. This
position is helpful in pooling venous blood in the periphery and reducing preload.
Assessing the patient’s oxygen saturation at rest and during activity may assist with
determining a need for supplemental oxygen. Oxygen is usually given through nasal
cannula when indicated. Ensuring that medications are taken as prescribed is important
to preserving adequate cardiac output. It is important to ensure that patients with HCM
avoid diuretics and that patients with DCM avoid verapamil (Calan, Isoptin) to maintain
contractility.
The nurse may assist the patient with planning a schedule for taking medications and
identifying methods to remember to follow it, such as associating the time to take a
medication with an activity (eg, eating a meal, brushing teeth). Ensuring that the patient
receives or chooses food selections that are appropriate for the low-sodium diet is also
important. Determining the patient’s weight every day and identifying any significant
change is one way to monitor the patient’s response to treatment. Patients with low
cardiac output may need assistance keeping warm and frequently changing position to
stimulate circulation and reduce the possibility of skin breakdown.
 INCREASING ACTIVITY TOLERANCE
The nurse plans the patient’s activities so that they occur in cycles, alternating rest with
activity periods. This benefits the patient’s physiologic status, and it helps to teach the
patient about the need for planned cycles of rest and activity. For example, after taking
a bath or shower, the patient should plan to sit and read the paper or pay bills.
Suggesting that patients sit while chopping vegetables, drying their hair or shaving helps
them to identify methods to balance rest with activity. The nurse can also make sure
that the patient recognizes the symptoms that indicate the need for rest and the actions
to take when the symptoms occur. Patients with HCM need to avoid strenuous activity
and sports.
 REDUCING ANXIETY
Spiritual, psychological, and emotional support may be indicated for the patient, family,
and significant others. Interventions are directed toward eradicating or alleviating
perceived stressors. The patient is provided with appropriate information about
cardiomyopathy and self-management activities. An atmosphere in which the patient
feels free to verbalize concerns is provided, as is assurance that these concerns are
legitimate. If the patient is facing death or awaiting transplantation, time must be
provided to discuss these issues. Providing the patient with realistic hope helps to
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reduce anxiety while the patient awaits a donor heart. Nurses help the patient, family,
and significant others with anticipatory grieving. Accomplishing a goal, no matter how
small, also promotes the patient’s sense of well-being.
 DECREASING THE SENSE OF POWERLESSNESS
Patients need to recognize that they go through a grieving process when given a
diagnosis of cardiomyopathy. They are assisted in identifying the things in their life that
they have lost (e.g., foods that they have enjoyed eating but are high in sodium, ability
to engage in constant active lifestyle, ability to play sports, ability to lift grandchildren).
They also are assisted in identifying their emotional responses to the loss (e.g., anger,
depression). The nurse then assists patients in identifying the amount of control that
they have in their lives, such as making food choices, managing their medications, and
working with their provider to achieve the best possible outcomes. The use of patient
tools that track behaviors with the resulting symptoms may be helpful. For example, a
diary in which the patient records his or her food selections and weight may assist the
patient with understanding the relationship between sodium intake and weight gain.
Some patients are able to manage a self-titrating diuretic regimen, in which the patient
is able to adjust the dose of diuretic to his or her symptoms.
 PROMOTING HOME AND COMMUNITY-BASED CARE
Teaching Patients Self-Care
Teaching patients about the medication regimen, symptom monitoring, and symptom
management is a key part of the plan of nursing care. The nurse is integral to the
learning process as patients learn to balance their lifestyle and work while
accomplishing their therapeutic activities. Helping patients cope with their disease
status assists them in adjusting their lifestyles and implementing a self-care program at
home.
Continuing Care
The nurse reinforces previous teaching and performs ongoing assessment of the
patient’s symptoms and progress. The nurse also assists the patient and family to adjust
to lifestyle changes. Teaching patients to read nutritional labels, to maintain a record of
daily weights and symptoms, and to organize daily activities to increase activity
tolerance can be helpful. The patient’s responses to diet and fluid restrictions and to the
medication regimen are assessed, and explanations about symptoms that should be
reported to the physician are emphasized. Because of the risk of dysrhythmia, the
patient’s family may be taught cardiopulmonary resuscitation. Women are often
advised to avoid pregnancy; each case is assessed individually. The nurse assesses the
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psychosocial needs of the patient and family on an ongoing basis. There may be
concerns and fears about the prognosis, changes in lifestyle, effects of medications, and
the possibility of others in the family having the same condition that increase the
patient’s anxiety and interfere with effective coping strategies. Establishing trust is vital
to the relationship with these chronically ill patients and their families. This is
particularly significant when the nurse is involved with the patient and family in
discussions about end-of-life decisions. Patients who have significant symptoms of heart
failure or other complications of cardiomyopathy may need a home care referral.
EVALUATION
EXPECTED PATIENT OUTCOMES
Expected patient outcomes may include:
1. Maintains or improves cardiac function
a. Exhibits heart and respiratory rates within normal limits
b. Reports decreased dyspnea and increased comfort; maintains or improves
gas exchange
c. Reports no weight gain
d. Maintains or improves peripheral blood flow
2. Maintains or increases activity tolerance
a. Carries out activities of daily living (e.g., brushes teeth, feeds self)
b. Reports increased tolerance to activity
3. Is less anxious
a. Discusses prognosis freely
b. Verbalizes fears and concerns
c. Participates in support groups if appropriate
4. Decreases sense of powerlessness
a. Identifies emotional response to diagnosis
b. Discusses the control he or she has in life
5. Adheres to the self-care program
a. Takes medications according to prescribed schedule
b. Modifies diet to accommodate sodium and fluid restriction
c. Modifies lifestyle to accommodate recommended activity and rest
behaviors
d. Identifies signs and symptoms to be reported to the health care
professional
CONCLUSION
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Cardiomyopathy is a cardiac disease process that results in heart failure due to a

decrease in the pumping power of the heart or due to impairment in the filling of the
cardiac chambers. Persons with cardiomyopathy frequently retain excess fluid, resulting
in congestion of the lungs, and have symptoms of weakness, fatigue, and shortness of
breath. Sometimes they develop a potentially fatal arrhythmia, or abnormal heart
rhythm.

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