PRESENTATION

ON

PULMONARY HYPERTENSION

Submitted to: submitted by:

Mrs.Ragini madam, k. vani

Government college of nursing, MSc (N) 1ST YEAR

Somajiguda, Government college of

Hyderabad nursing,

Somajiguda.
 STUDENT PROFILE

Name of the subject : Medical surgical nursing –

CT specialty

Name of the topic : pulmonary hypertension

Name of the student : K. Vani

Unit : VI

Time : 3hour

Place : M.Sc. (N) II nd year class room

Group : M.Sc. (N) II nd year(7 students)

Method of teaching : lecturer cum discussion method

Av Aids : power point presentation and

hand outs

Name of the guide : Mrs. Ragini madam

Name of the HOD : Mrs.DR.K.T.Sheeba Madam

 OBJECTIVES

General objectives: - by, the end of the presentation, the group will be
able to gain in depth knowledge regarding pulmonary hypertension.

Specific objectives: -

At the end of the presentation the group will be able to: -

 Review of pulmonary circulation.

 Define pulmonary hypertension.
 Discuss about the history of pulmonary hypertension.
 Enlist the incidence of pulmonary hypertension (globally, India and
Telangana).
 Enumerate the classification of pulmonary hypertension.
 Explain about the etiology of pulmonary hypertension.
 Discuss about the clinical manifestations of pulmonary hypertension.
 Enlist the diagnostic finding of pulmonary hypertension.
 Explain about the medical management.
 Enumerate about the surgical management.
 Discuss about the complementary/alternative therapies of pulmonary
hypertension.
 Explain about the nursing management of pulmonary hypertension.
 List out the complication of pulmonary hypertension.
 Discuss about the prevention of pulmonary hypertension.
 Discuss about the prognosis of pulmonary hypertension.
 Summarize the topic.
 INTRODUCTION

The human body is made of 75% of water in the form of blood,

lymph etc. Blood is a vitally important fluid for the body. A man who
weighs about 70 kg (about 154 pounds) has about 5 to 6 liters of blood in his
body. Blood has three important functions: The blood transports oxygen
from the lungs to the cells of the body, where it is needed for metabolism.
The carbon dioxide produced during metabolism is carried back to the lungs
by the blood, where it is then exhaled (breathed out). Blood also provides the
cells with nutrients, transports hormones and removes waste products, which
organs such as the liver, the kidneys or the intestine then get rid of. Blood
pressure (BP) is the pressure of circulating blood against the walls of blood
vessels. Most of this pressure results from the heart pumping blood through
the circulatory system.

Pulmonary hypertension is a rare lung disorder in which the arteries that

carry blood from the heart to the lungs become narrowed, making it difficult
for blood to flow through the vessels. As a result, the blood pressure in these
arteries -- called pulmonary arteries -- rises far above normal levels. This
abnormally high pressure strains the right ventricle of the heart, causing it to
expand in size. Overworked and enlarged, the right ventricle gradually
becomes weaker and loses its ability to pump enough blood to the lungs.
This could lead to the development of right heart failure. Pulmonary
hypertension (PH) is an abnormal elevation in pulmonary artery pressure, it
is a feature of advanced disease. The pulmonary artery pressure and
pulmonary vascular resistance progressively rises, leading to right heart
failure and death. Over the years, improvement in understanding the
pathogenesis has resulted in the development of targeted approaches to the
treatment of PH. Survival advantage has also been shown with some of the
pharmacologic agents.

REVIEW OF ANATOMY AND PHYSIOLOGY OF PULMONARY

CIRCULATION:

The pulmonary circulation is the portion of the circulatory system

which carries deoxygenated blood away from the right ventricle, to the lungs,
and returns oxygenated blood to the left atrium and ventricle of the heart.
The term pulmonary circulation is readily paired and contrasted with the
systemic circulation. The vessels of the pulmonary circulation are the
pulmonary arteries and the pulmonary veins.

ANATOMY

Lungs have 3 circulation.

 Pulmonary circulation
 Bronchial circulation
 Lymphatic circulation.

PULMONARY CIRCULATION

Pulmonary trunk

Right & left pulmonary artery

Right & left lungs

Capillaries lining of alveoli

 Get oxygenated & return back via pul veins to left atrium.

BRONCHIAL CIRCULATION

 Descending thoracic aorta give right & left bronchial arteries

 Supply oxygenated blood to lungs (connective tissue, septa & bronchi)
& after joins pulm veins without (Bypass) oxygenation, so forms
Physiological shunt.

LYMPHATIC CIRCULATION.

Present in walls of terminal bronchioles & supportive tissues of lung.

Removes particulate matter, plasma proteins – thus prevents pulmonary
oedema

Drainage pathway

Deep lymphatic

Pulmonary nodes

Bronchopulmonary nodes

Tracheobronchial nodes

Broncho mediastinal trunk.

PULMONARY CIRCULATION CHARACTERISTIC FEATURES.

 Thickness of Right ventricle and pulmonary artery 1/3rd of left

ventricle & aorta.
 Pulmonary circulation is low pressure, low resistance & high
capacitance system.
 Pulmonary capillaries are larger in diameter than systemic capillaries.
 Each alveolus is enclosed in basket of capillaries.
PRESSURES IN PULMONARY SYSTEM.

1. Right ventricular pressure.

2. Pulmonary artery pressure.
3. Left atrial pressure.
4. Pulmonary capillary pressure.

Right ventricular pressure: During each cardiac cycle,

During Systole – reaches peak 25 mm Hg. (120 mm Hg in Left ventricle)

During Diastole – 0-1 mm Hg (5 mm Hg in left ventricle)

Pulmonary artery pressure

Systolic pressure 25 mm Hg (120 mm Hg in Aorta)

Diastolic pressure 8 mm Hg (8 mm Hg in Aorta)

Mean arterial pressure 15 mm Hg (100 mm Hg in Aorta)

Pulse pressure 17 mm Hg (40 mm Hg in Aorta)

Left atrial pressure

Major pulmonary veins pressure avg 5 mm Hg

So Pressure gradient in pulmonary system is

Mean pulmonary artery pressure – mean pulmonary vein pressure

15-5 = 10 mm Hg.

Pulmonary capillary pressure

10 mm Hg.
Colloidal osmotic pressure is 25 mm Hg, So net suction force of 15 mm Hg
draw fluid from pulmonary interstitial fluid into pulmonary capillary, So
keeps Alveoli dry

SIGNIFICANCE OF LOW PULMONARY CAPILLARY PRESSURE

So if pulmonary capillary pressure rises above 25 mm Hg, Fluid escapes

into interstitial spaces, Lead to pulmonary oedema, Conditions raising this
pressue, Exercise at high altitude, Left heart failure, Mitral stenosis and
Pulmonary fibrosis.

PULMONARY BLOOD VOLUME

Pulmonary vessels contain – 600 ml; its capacitance vary from 200-900 ml
Pulmonary blood volume decreases during standing & during haemorrhage
to compensate, so acts as Reservoir.

PULMONARY BLOOD FLOW

Pulmonary blood flow nearly equal to cardiac output. Blood flow through
lung depend on Relationship between pressures of Pulmonary artery,
pulmonary vein & alveolar artery.

REGULATION OF PULMONARY BLOOD FLOW.

Neural control.

 Efferent sympathetic vasoconstrictor nerves: Innervates pulmonary

blood vessels and Participate in vasomotor reflexes.

Baroreceptor stimulation – causes reflex dilatation of pulmonary

vessels

Chemoreceptor stimulation – causes pulmonary vasoconstriction.

  Afferent control through vagus is mediated through receptors.
 Pulmonary baroreceptors
 pulmonary volume receptors
 J receptors.

CHEMICAL CONTROL

Local Hypoxia – causes change in blood flow by vasoconstriction.

Hypercapnia & acidosis – causes vasoconstriction.(Vasod ilatation in
systemic circulation)

Chronic Hypoxia: Occurs in high altitude dwellers associated with

pulmonary hypertension followed by right ventricular hypertrophy, right
heart heart failure & pulmonary oedema.

:DEFINITIONS:

 Pulmonary arterial hypertension (PAH) is defined as a mean

pulmonary artery (PA) pressure of 2:25 mm Hg at rest.

- BY Ashok Seth (advanced cardiology medicine textbook).

 Pulmonary hypertension is a condition that is not clinically evident

until late in its progression. Pulmonary hypertension exists when the
systolic pulmonary artery pressure exceeds 30 mm Hg or the mean
pulmonary artery pressure exceeds 25 mm Hg. _

BY burner and Siddharth

 Pulmonary hypertension is characterized by elevated pulmonary

artery pressure, resulting from an increase in resistance to blood flow
through the pulmonary circulation. by Lewis.
  Pulmonary hypertension exists when the systolic pulmonary artery
pressure exceeds 30mm Hg or the mean pulmonary artery pressure
exceeds 25mm Hg (Smeltzer SC, Bare BG)
 Pulmonary hypertension is defined as a prolonged elevation of the
mean pulmonary artery pressure above 25 mm Hg at rest (normal 10
to 20 mm Hg) or above 30 mm Hg during exercise (normal 20 to 30
mm Hg) (Black JM, Hawks JH)

HISTORY:

 E. Romberg, German doctor published description of autopsy, showed

thickening of the pulmonary artery but no heart or lung disease.
 In 1951, 39 cases were reported by Dr. D.T. Dresdale in the United
States.
 Between 1967 and 1973, a 10-fold increase in unexplained PH was
reported in central Europe.
 The rise was subsequently traced to Aminorex fumarate, an
amphetamine-like drug introduced in Europe in 1965 to control
appetite.

INCIDENCE:

Globally:

Pulmonary hypertension is an under-recognized global health issue and is by

no means rare. pulmonary hypertension prevalence of about 1% of the
global population, which increases up to 10% in individuals aged more than
65 years. In almost all parts of the world, left-sided heart and lung diseases
have become the most frequent causes of pulmonary hypertension
In economically developed countries, left-sided heart disease and lung
disease are by far the most common causes of pulmonary hypertension.

in industrialized countries, pulmonary hypertension affects mainly elderly

people whereas mostly young people are diagnosed in the developing world.

India:

estimates of the incidence of pulmonary arterial hypertension range from

one to two cases per million in the general population. The incidence of
PAH in portal hypertension or in HIV infection has been estimated to
bebetween 0.5% and 2%. (lung India article)

CLASSFICATION:

Based on the origin:

there are two forms of pulmonary hypertension:

primary (or idiopathic): Primary pulmonary hypertension is an uncommon

disease in which the diagnosis is made by excluding all other possible causes.

 Altered immune mechanisms

 Silent pulmonary emboli
 Raynaud’s phenomenon
 Oral contraceptive use
 Sickle cell disease
 Collagen diseases

Secondary: Secondary pulmonary hypertension is more common and

results from existing cardiac or pulmonary disease.

 Pulmonary vasoconstriction due to

  hypoxemia
 Chronic obstructive pulmonary disease
 Kyphoscoliosis
 Obesity
 Smoke inhalation
 High altitude
 Neuromuscular disorders
 Diffuse interstitial pneumonia
 Reduction of the pulmonary vascular bed (must impair 50% to 75% of
the vascular bed)
 Pulmonary emboli
 Vasculitis
 Widespread interstitial lung disease (sarcoidosis, systemic
sclerosis)
 Tumor emboli
 Primary cardiac disease
 Congenital (patent ductus arteriosus, atrial septal defect,
ventricular septal defect).
 Acquired (rheumatic valvular disease, mitral stenosis, myxoma,
left ventricular failure)

CLASSIFICATION BASED ON ETIOLOGY:

The World Health Organization (WHO) classifies patients with PH into five
groups based upon etiology: Dana Point, 2008.

 Group 1: Pulmonary arterial hypertension (PAH). it is further divided

into the following 4 subgroups:
  Subgroup 1 - Idiopathic PAH (IPAH)
 Subgroup 2 - Heritable PAH
 Subgroup 3 - Drug- and toxin-induced PAH
 Subgroup 4 - Conditions with known localization of lesions in
the small pulmonary arterioles, including collagen-vascular
disease
 Subgroup 5 – Persistent pulmonary hypertension of the
newborn.
 Group 2: Pulmonary hypertension due to left heart diseases.
 Group 3: Pulmonary hypertension due to lung diseases and/or
hypoxemia.
 Group 4: Chronic thromboembolic pulmonary hypertension
(CTEPH).
 Group 5: Pulmonary hypertension with unclear multifactorial
mechanisms.

BASED ON SIGNS AND SYMPTOMS:

NEW YORK HEART ASSOCIATION AND WORLD HEALTH ORGANIZATION
FUNCTIONAL CLASSES IN PULMONARY HYPERTENSION.

Class Symptoms/Function
No limitation in physical activity.
NYHA I / WHO I Ordinary physical activity does not cause undue
dyspnoea, fatigue, chest pain, or near-syncope.
Slight limitation in physical activity.
NYHA II / WHO II Ordinary physical activity causes undue dyspnoea,
fatigue, chest pain, or near-syncope.
NYHA III / WHO III Marked limitation in physical activity.
Less than ordinary physical activity causes undue
dyspnoea, fatigue, chest pain, or near-syncope.
Inability to carry out any physical activity without
NYHA IV / WHO IV symptoms.
Patients manifest signs of right heart failure.
Dyspnoea and/or fatigue may be present at rest.
ETIOLOGY:

Pulmonary hypertension is a pathophysiologic condition with many possible

causes. Indeed, this condition frequently accompanies severe heart or lung
condition

Group 1: Pulmonary arterial hypertension (PAH):

 Idiopathic: PAH corresponds to sporadic disease, without any familial

history of PAH or known triggering factor. Formerly referred to as
primary pulmonary hypertension 1-2 cases per million
Females>males
 Genetic: BONE MORPHOGENETIC PROTEIN RECEPTOR 2
GENE(BMPR2) a type 2 receptor for bone morphogenetic proteins,
which belong to the TGF-b superfamily involved in the control of
vascular cell proliferation. mutations are detected in at least 70% of
cases PAH occurs in a familial context
 Drug induced: most of the drug induced pulmonary hypertension is
due to alteration in serotonin (it is a neuro transmitter) metabolism in
humans, serotonin can enter PASMCs via the serotonin transporter
(SERT) or activate the 5-HT1B receptor; 5-HT1B activation and
 SERT activity which results in mediate proliferation, vasoconstriction,
and fibrosis in the pulmonary circulation.
 Opioids and substances of abuse: Buprenorphine and cocaine
have been identified as potential causes of pulmonary arterial
hypertension. The mechanism by which this occurs is unclear.
Tramadol has been demonstrated to cause severe, transient, and
reversible pulmonary hypertension.
 Chemotherapeutic agents: Alkylating and alkylating-like
agents, such as bleomycin, cyclophosphamide, and mitomycin
have increased the risk of pulmonary veno-occlusive disease.
Thalidomide and paclitaxel have also been implicated as
potential causes.
 Miscellaneous medications: Protamine appears to be able to
cause acute, reversible pulmonary hypertension when bound to
heparin. Amiodarone is also capable of causing pulmonary
hypertension.
 Persistent pulmonary hypertension of the newborn (PPHN): is most
commonly associated with 1 of 3 underlying etiologies: Acute
pulmonary vasoconstriction. Hypoplasia of the pulmonary vascular
bed (commonly seen with congenital diaphragmatic hernia) Idiopathic
pulmonary hypertension.

Group 2: Pulmonary hypertension due to left heart diseases.:

Pulmonary hypertension due to left heart diseases is Characterized by

enlarged and thickened pulmonary veins, pulmonary capillary dilatation,
interstitial oedema, alveolar hemorrhage, and lymphatic vessel and lymph
node enlargement. Distal pulmonary arteries will be affected
Left heart condition includes:

 Systolic dysfunction
 Diastolic dysfunction
 Valvular disease

Group 3: Pulmonary hypertension due to lung diseases and/or

hypoxemia

-Pulmonary hypertension due to lung diseases and/or hypoxia Include

medial hypertrophy and intimal obstructive proliferation of the distal
pulmonary arteries. A variable degree of destruction of the vascular bed in
emphysematous or fibrotic areas may also be present.

Causes include:

 Chronic obstructive pulmonary disease (COPD)

 Interstitial lung disease (ILD)
 Other pulmonary diseases with mixed restrictive and obstructive
pattern
 Sleep-disordered breathing
 Alveolar hypoventilation disorders
 Chronic exposure to high altitude
 Developmental abnormalities

Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH).

Characterized by organized thrombi tightly attached to medial layer in

pulmonary arteries, replacing the normal intima. These may completely
occlude the lumen or form different grades of stenosis, webs, and bands.
Collaterals from the systemic circulation can grow.
Group 5: Pulmonary hypertension with unclear multifactorial
mechanisms.

In association with a systemic disease where a causal relationship is not

clearly understood. Includes:

 Hematological disorders: myeloproliferative disorder, splenectomy)

Hemolysis observed in hematological disorders leads to free
hemoglobin which rapidly scavenges nitric oxide (NO), limiting its
bioavailability, and leading to endothelial dysfunction.
 Systemic disorders: sarcoidosis, pulmonary Langerhans cell
histiocytosis, neurofibromatosis, vasculitis (The mechanism for
development of pulmonary hypertension may be related to LV
geometry and remodeling, and consequent changes in diastolic
function.)
 Metabolic disorders: Glycogen storage disease, Gaucher disease
Thyroid disorders(metabolic dysfunction in PAH, as alterations in
aerobic glycolysis, fatty acid oxidation (FAO), and the tricarboxylic
acid (TCA) cycle have been associated with PAH and the
development of lipotoxicity in the RV.
 Thyroid: Both hyperthyroidism and hypothyroidism produce changes
in cardiac contractility, myocardial oxygen consumption, cardiac
output, blood pressure, and systemic or pulmonary vascular resistance.
Possible mechanisms include:

1) enhanced catecholamine sensitivity, causing pulmonary

vasoconstriction, a reduction in pulmonary vascular compliance and
an increase in vascular resistance;
 2) increased metabolism of intrinsic pulmonary vasodilating
substances (prostacyclin, nitric oxide);

3) decreased or impaired metabolism of vasoconstrictor's (serotonin,

endothelin 1 and thromboxane). In some cases (Graves's and
Hashimoto's disease) and an autoimmune process inducing endothelial
damage may play a key role.

 Others: Tumoral obstruction, fibrosing mediastinitis chronic renal

failure on dialysis (as it alters the hemodynamic state of the body)
 Prostacyclin: Prostacyclin is a potent vasodilator, inhibits
platelet activation, and has antiproliferative properties.
 Nitric Oxide: Nitric oxide (NO) is a vasodilator and inhibitor
of platelet activation and vascular smooth-muscle cell
proliferation.
 Serotonin (5-HT) is a vasoconstrictor and promotes PASMC
hypertrophy and hyperplasia. Allelic variation in serotonin
transporter present in PA Vasoactive intestinal peptide (VIP)
has a pharmacologic profile similar to prostacyclin's.

RISK FACTORS:

Growing older can increase your risk of developing pulmonary hypertension.

The condition is more often diagnosed in people ages 30 to 60. However,
idiopathic PAH is more common in younger adults.

Other things that can raise your risk of pulmonary hypertension include:

 family history: gene mutation leads to the pulmonary hypertension

 Obesity and obstructive sleep apnea. In isolation, obesity is not a
risk factor. However, if obesity is combined with obstructive sleep
apnea (meaning that oxygen levels fall while a person is sleeping),
mild PH may occur.
 Gender. Idiopathic PAH and heritable PAH (also known as familial
PAH) are at least two-and-a-half times more common in women than
in men. Females of childbearing age are also more susceptible.
 Pregnancy. The leftward shift of the interventricular septum increases,
impairing diastolic filling of the left ventricle and further
compromising cardiac output. Moreover, the hypercoagulability of
pregnancy increases the risk of pulmonary emboli and pulmonary
arterial thrombosis.
 Altitude. Living at a high altitude for years can make more
predisposed to pulmonary hypertension. It leads to low oxygen levels
and leads to increase the nitric oxide which leads to vasoconstriction
and vascular dysfunction.
 Other diseases. Other diseases, including congenital heart disease,
lung disease, liver disease and connective tissue disorders like
scleroderma and lupus, can lead to the development of pulmonary
hypertension.
 Drugs and toxins. Certain drugs, such as methamphetamines and the
diet drug “fen phen,” are known to cause pulmonary hypertension.
PATHOPHYSIOLOGY:

Etiology/ causes

Pulmonary vasoconstriction

Vascular obstruction

Increased blood flow

Loss of pulmonary vascular bed

pulmonary vasoconstriction: due to hypoxia and, to a lesser extent, to

acidosis. In The muscular arteries show hypertrophy of the smooth muscle in
media layer, and hyperplasia of the intimal layer lining the vessel lumen.

In the arterioles, a significant muscular component to the vessel wall

normally is not present, but with pulmonary hypertension these vessels
undergo “remuscularization” of their walls. AND arteriolar intima
proliferates.

As a result of these changes, the luminal diameter is significantly decreased,

and the pulmonary vascular resistance is elevated. Ultimately, the lumen
may be completely obliterated and the overall number of small vessels
greatly diminished. the small arterioles may demonstrate so-called plexiform
changes, appearing as a plexus of small, slit like vascular channels.
vascular obstruction: emboli, pulmonary artery stenosis: as changes in the
diameter of vessels is decreased and plexiform changes there will be
obstruction in the normal blood flow.

increased blood flow (e.g., left-to-right intracardiac shunts—atrial and

ventricular septal defects): increased pulmonary vascular resistance and right
ventricular pressure. As the architectural changes of pulmonary hypertension
progress, both right ventricular and pulmonary arterial pressures rise because
of increased pulmonary vascular resistance. Cardiac output usually remains
normal early in the course of the process. When the right ventricle fails, right
ventricular end-diastolic pressure rises, and cardiac output may decrease as
well. Right atrial pressure also rises

loss of pulmonary vascular bed the total cross- sectional area of the
pulmonary vascular bed is compromised by parenchymal lung disease, with
loss of blood vessels from either a scarring or a destructive process affecting
the alveolar walls. (e.g., fibrotic lung disease, emphysema).

CLINICAL MANIFESTATIONS:

The symptoms of pulmonary hypertension include the follow

 Dyspnea: because “the right side of the heart is having trouble

pushing blood flow through the lungs and it’s not getting to the left
side of the heart and body, it puts strain on the right side of the heart,
which is not used to pushing against the high pressure.” and oxygen
deprivation also leads to shortness of breath.
 Fatigue: it is due to lack or decreased oxygen supply
 Angina: Angina, a common symptom that is often underappreciated,
most often results from right ventricular ischemia (but can result from
 left ventricular ischemia secondary to compression of the left main
coronary artery by an enlarged main pulmonary artery).
 Palpitations (heartbeat rate increased): Constricted and narrowed
arteries prevent your heart from pumping adequate blood. When
constriction occurs, the heart will need to work harder to compensate.
This causes the blood pressure in the pulmonary arteries and in the
heart to increase dramatically.
 Right-sided abdominal pain: it is a late symptom of pulmonary
hypertension due to right side dysfunction or mitral valve dysfunction.
 Poor appetite: the exact rational is unknown.
 Lightheadedness: lack or deprivation of oxygen supply.
 Swelling (legs/ankles): as pulmonary hypertension causes the right-
side heart dysfunction, so there will be alteration in the venous
drainage from the different parts of the body resulting in lacking
blood backflow to heart leads to leg edema. Edema is generally a
reflection of right ventricular failure and is more likely to be
associated with advanced pulmonary vascular disease.
 Cyanosis : deprived oxygenation of blood.
 A loud pulmonic valve closure (P2) or an early systolic ejection
click may be heard as a consequence of marked elevation in PA
pressures. With right ventricular hypertrophy and enlargement, a left
parasternal lift can be palpated. Another manifestation of right
ventricular hypertrophy with diastolic dysfunction is the right
ventricular S4 gallop. A right-sided S3 is also appreciable in patients
with significant elevation in the right ventricular end-diastolic
pressure.
  Murmur: Often the holosystolic murmur of tricuspid regurgitation
(although frequently without the classically described respiratory
variation) and the less common diastolic murmur of pulmonic
insufficiency are noted in patients with PH. Jugular venous distention,
peripheral edema, and ascites become manifest in advanced PH with
right heart failure. Cool extremities and diminished peripheral pulses
are indicative of low cardiac output and peripheral vasoconstriction in
severe right ventricular failure.
 A murmur of mitral or aortic stenosis, a left-sided S3, or severe
bronchial wheezing and diminished breath sounds may be clues to the
presence of left-sided heart disease or pulmonary parenchymal disease,
respectively. Jaundice and spider angiomas may point to the presence
of cirrhosis and portal hypertension. Connective tissue diseases may
present not only with signs of PH but also with Raynaud phenomenon,
arthritis, rashes or other skin changes (i.e., sclerodactyly).
 Pulmonary venous hypertension typically presents with shortness of
breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal
dyspnea).

GRADING:

SYSTOLIC DIASTOLIC MEAN

GRADE 1(mild) 30-50 20-25 >30
GRADE 2 50-70 26-35 >40
(moderate)
GRADE 3 70-110 36-45 >50
(severe)
GRADE 4 >110 46-55 >60
(systemic or
spura systemic)
DIAGNOSTIC EVALUATION:

Because pulmonary hypertension may be caused by many medical

conditions, a complete medical history, physical exam, and description of
symptoms is necessary to rule out other diseases and make the correct
diagnosis.

HISTORY COLLECTION:

In the history collection includes the:

Biographic data: occupation and living atmosphere are given priority.

Present medical history: each and every symptom with the characteristics are
cleared assessed.

Patients should be asked to describe their pain in terms of the following

characteristics: location, radiation, mode of onset, character, temporal
pattern, exacerbating and relieving factors, and intensity.

Describe dyspnea whether it Is all the time or presents with walking or doing
daily activities. aggravating and relieving factors.

Past history: any recent pulmonary or heart disease conditions, thyroid, liver
diseases. Diabetes, hypertension and any other systemic disease conditions.
Family history of pulmonary hypertension and usage of medications should
be assessed

Life style and personal history: information regarding alcohol, smoking

habits, exercise pattern, food preference, amount of daily fat intake and
water intake, occupation, sleeping pattern should be collected.
PHYSICAL EXAMINATION:

During the physical exam,

♦ Listen for abnormal heart sounds such as a loud pulmonic valve

sound, a systolic murmur of tricuspid regurgitation, or a gallop due to
ventricular failure.
♦ Examine the jugular vein in the neck for engorgement (jugular vein
distension)
♦ Examine the abdomen, legs, and ankles for fluid retention(edema).
♦ Examine nail beds for bluish tint indicates cyanosis.

BLOOD INVESTIGATIONS:

 Complete metabolic panel (CMP): Examines liver and kidney

function
 Autoantibody blood tests, such as ANA, ESR, and others: Screens
for collagen vascular diseases
 Thyroid stimulating hormone (TSH): A screen for thyroid problems
 HIV: A screen for human immunodeficiency virus
 Arterial blood gases (ABG): Determines the level of oxygen in
arterial blood.
 Complete blood count (CBC): Tests for infection, elevated
hemoglobin, and anemia
 B-type natriuretic peptide (BNP): A marker for heart failure.
RADIOLOGICAL INVESTIGATIONS:

1. Electrocardiography

The electrocardiogram (ECG) in patients with significant PH typically

suggests right ventricular hypertrophy. There is often a distinct correlation
between the amplitude of the R wave in V1, the R/S ratio in V1, and the
severity of the PH. Classic ECG findings include right axis deviation and
right atrial enlargement. Incomplete or complete right bundle branch block is
also common. The ECG is not sensitive enough to serve as a screening tool
for PH.

2. Chest radiography

Radiographic examination of the chest in a patient with PAH may

show enlargement of the main pulmonary artery and its major branches, with
distal pruning of peripheral arteries. The retrosternal space is often filled by
the hypertrophic right ventricle on lateral projection. Pulmonary venous
congestion, left atrial or left ventricular enlargement suggest the presence of
a left-sided cause of PH. Hyperinflated lung fields or bullous changes point
to a chronic pulmonary disorder with secondary PH.

3. Echocardiography

Two-dimensional and Doppler echocardiography are essential to the

noninvasive assessment of patients with suspected PH. Echocardiography
can demonstrate structural changes such as right atrial or right ventricular
enlargement, right ventricular hypertrophy, and PA enlargement. Flattening,
or leftward shift of the interventricular septum can also be identified. If the
shift occurs during systole, it suggests ventricular pressure overload. If it
occurs during diastole, it suggests volume overload. Leftward shift of the
septum throughout the cardiac cycle suggests both right ventricular pressure
and volume overload. The left ventricle is often small and underfilled in
PAH, with normal systolic function.

Tricuspid regurgitation can also be assessed by echocardiography.

Using the velocity of the tricuspid regurgitant jet and inferior vena cava
dynamics, pulmonary artery systolic pressure (PASP) can be estimated using
the modified Bernoulli equation (PASP = 4v2 + right atrial [RA] pressure).
Noninvasive PA pressure assessment is not only important in establishing a
diagnosis of PH but also in monitoring the patient’s response to therapy.

Echocardiography can also be helpful in detecting left- sided heart

disease. A dilated, hypo contractile left ventricle, valvular disease, or left
atrial myxoma can be identified or excluded on a routine echocardiogram.
Color-flow and agitated saline injection can also be used to assess for the
existence of congenital intracardiac or intrapulmonary shunts.

4. Lung scintigraphy

Ventilation-perfusion lung scintigraphy is an important screening tool

in the evaluation of PH due to chronic thromboembolic disease. In PAH, the
lung scan may reveal a normal perfusion pattern or it may show diffuse,
patchy (“mottled”) perfusion defects. Parenchymal lung disease can also
result in perfusion scan abnormalities, but typically, these are matched by
ventilatory defects. In chronic thromboembolic PH, however, the lung
perfusion scan demonstrates one or more segmental defects mismatched by
the ventilation scan. An abnormal ventilation-perfusion scan should prompt
further testing with a spiral CT scan or pulmonary angiography.
5. Pulmonary function testing

Pulmonary function testing is helpful in PH because it can establish

the diagnosis of underlying obstructive or restrictive pulmonary disease.
Interpretation of pulmonary function test results should be tempered by an
awareness that PAH can reduce diffusion capacity. If the total lung capacity
is less than 70% of predicted, a high-resolution CT scan should be
considered to evaluate for ILD.

6. Computed tomography and magnetic resonance imaging

CT scans and magnetic resonance imaging (MRI) of the chest can

provide useful information in parenchymal lung disorders and several other
conditions that can cause PH. Chest CT or MRI scans can demonstrate
characteristic findings in patients with fibrosing mediastinitis and cystic
fibrosis as well as infiltrative or granulomatous lung diseases.

7. Cardiac catheterization and pulmonary angiography

Right heart catheterization represents the gold-standard test to

establish the diagnosis of PH, ascertain its etiology, establish severity and
prognosis, evaluate vasoreactivity, and assist in guiding therapy.

At right heart catheterization, a full cardiopulmonary hemodynamic

profile is established by recording the right atrial, right ventricular, PA, and
pulmonary arterial wedge pressures as well as measuring the CO. The PVR
(mean PA — PAWP/CO) is also determined during right heart
catheterization. Arterial and venous oxygen saturations obtained during right
heart catheterization are used to detect and determine the severity of shunts.
Abnormalities of the left heart, such as aortic or mitral valve disease or left
heart failure, will be suggested during right heart catheterization by the
presence of an elevated PAWP.

Vasoreactivity testing is an important component of right heart

catheterization in PAH. Acute vasoreactivity is tested using inhaled nitric
oxide or intravenous prostacyclin or adenosine. A reduction in mean PA
pressure> 10 mm Hg with an absolute mean PA pressure ≤ 40 mm Hg
suggests that reversible vasoconstriction, rather than fixed vascular
remodeling, is the major mechanism for PAH. Less than 15% of patients
with idiopathic PAH will respond to acute vasodilator testing, but a positive
vasoreactivity trial identifies a subset of patients with superior overall
prognosis and greater likelihood of benefit from calcium channel blocker
therapy.

Pulmonary angiography is not routinely conducted a patient with PH.

When ventilation-perfusion scintigraphy or a spiral CT scan suggests
chronic thromboembolic disease, pulmonary angiography and angioscopy
can determine the extent and location of thromboembolic disease. For this
reason, pulmonary angiography is often used as part of the preoperative
assessment of patients considered for thromboendarterectomy.

8. Lung biopsy

A lung biopsy is infrequently required to determine the cause of PH.

Lung biopsy can identify injected particulate matter in injection drug users,
establish the diagnosis of pulmonary veno-occlusive disease, and may
identify causative pathogens in ILD. Due to its substantial morbidity,
however, lung biopsy is seldom used. If 1ung biopsy is obtained in an
individual with reversible PH, medial smooth muscle hypertrophy may be
noted. In more advanced cases, necrotizing arteritis and plexiform lesions
can be seen.

9. Functional capacity and exercise testing

The use of World Health Organization’s functional classification and

symptom-limited exercise testing can be very helpful in the evaluation of
patients with PH. Exercise testing, particularly the 6-minute walk test, has
been shown to predict mortality and allows for objective assessment of
symptom burden. Baseline functional capacity and exercise testing utilizing
the 6-minute walk test should be measured prior to initiation of therapy and
serially during treatment.

Differential Diagnoses
Apnea, Sleep
Arteriovenous Malformations
Atrial Myxoma
Atrial Septal Defect
Cardiomyopathy, Dilated, Hypertrophic, Restrictive
Chronic Obstructive Pulmonary Disease, Emphysema
Mitral Regurgitation, Mitral Stenosis
Pulmonary Hypertension, Primary
Restrictive Lung Disease
Systemic Lupus Erythematosus.
MANAGEMENT

Treatment of pulmonary hypertension depends on an accurate

assessment of the cause of the pulmonary hypertension.
GENERAL MEASURES

Important general measures for patients with all forms of pulmonary

arterial hypertension include the avoidance of circumstances or substances
that may aggravate the disease state.

TREATMENT OF UNDERLYING CONDITIONS

The first-line treatment should be directed at any underlying or

associated conditions. After these other disorders have been optimally
treated, specific treatment of the pulmonary arterial hypertension itself
should be considered.

CONVENTIONAL MEDICAL THERAPY

Anticoagulation

Histologic data demonstrate thrombotic lesions in small pulmonary

arteries in a significant percentage of patients with idiopathic pulmonary
arterial hypertension, and limited clinical data support the chronic use of
anticoagulation in patients with idiopathic pulmonary arterial hypertension.

Warfarin anticoagulation is recommended to achieve an international

normalized ratio (INR) of 1.5 to 2; however, certain clinical circumstances
may require a higher INR, and a lower INR is often appropriate for patients
at higher risk for bleeding.

Heparin (5000 to 10,000 U subcutaneously twice daily) or low-

molecular-weight heparin (1 mg/kg subcutaneously twice daily) may be
suitable alternatives in patients with adverse effects from warfarin.

Patients with chronic thromboembolic disease are treated with higher

doses of warfarin (i.e., to achieve an INR of 2.5 to 3.5).
 Whether chronic anticoagulation is useful in patients with other forms
of pulmonary arterial hypertension remains unknown.

Calcium-Channel Blockers

Calcium channel blocker therapy may reduce PA pressure and right

ventricular afterload, thereby improving the right ventricular hemodynamics
and increasing cardiac output. Approximately 7% of adult patients with
idiopathic pulmonary arterial hypertension appear to have a favorable
respond to chronic oral calcium-channel blockade, documented by an
improvement in symptoms, exercise tolerance, hemodynamics, and survival.

Although most studies have used calcium-channel blockers at

relatively high doses, such as long-acting nifedipine, 120 to 240 mg daily, or
amlodipine, 20 to 40 mg daily, the optimal dosing for patients with
idiopathic pulmonary arterial hypertension is uncertain. Verapamil is
contraindicated due to its significant negative inotropic properties. Use of a
calcium channel blocker is not advisable in patients with severe right
ventricular failure or hypotension.

inotropic Agents/Diuretics

The efficacy and toxicity of cardiac glycosides in patients with

pulmonary arterial hypertension remain unknown. Digoxin may help
improve right sided hemodynamics and can be an effective rate controlling
agent in the setting of atrial tachyarrhythmia. Diuretics can reduce the
increased intravascular volume and hepatic congestion that occur in patients
with right heart failure, although great care should be taken to avoid the
excessive diuresis that can decrease cardiac output in patients who are highly
dependent on preload.
SUPPLEMENTAL OXYGEN

Supplemental low-flow oxygen alleviates the arterial hypoxemia and

attenuates the pulmonary hypertension in patients with chronic pulmonary
parenchymal disease. In contrast, most patients with the Eisenmenger
syndrome derive little hemodynamic benefit from supplemental oxygen,
although all patients may benefit from supplemental ambulatory oxygen if
they experience oxygen desaturation with activity.

TARGETED MEDICAL THERAPY

Prostaglandins

Prostacyclin (epoprostenol) or prostacyclin analogue treatment is

supported by the imbalance of thromboxane to prostacyclin and the
demonstration of a reduction in prostacyclin synthase in the pulmonary
arteries of patients with idiopathic pulmonary arterial hypertension.
Continuous intravenous epoprostenol improves exercise endurance and
hemodynamics in patients with functional class III or IV idiopathic
pulmonary arterial hypertension or pulmonary arterial hypertension related
to connective tissue disease in addition, survival is improved with
continuous intravenous epoprostenol in patients with functional class III or
IV idiopathic pulmonary arterial hypertension.

The starting dose is 1 to 2 ng/ kg/ min with incremental increases,

especially during the first several months of initiation. A mean dose after 1
year is 20 to 40 ng/ kg/ min for most patients, although there appears to be
significant variability in the optimal dose. Continuous intravenous
epoprostenol has also been used to treat patients with pulmonary arterial
hypertension related to congenital systemic-to-pulmonary shunts, portal
hypertension, HIV infection, or drugs and toxins, with reported
improvement in exercise capacity, hemodynamics, and possibly survival in
uncontrolled studies.

In an attempt to avoid intravenous therapy, prostacyclin analogues

administered by continuous subcutaneous infusion (Treprostinil) or by
inhalation (iloprost; six to nine inhalations per day; 2.5 to 5 µg per dose) are
alternatives. Treprostinil is started at 1 ng/ kg/ min and increased to achieve
an optimal dose; Treprostinil is approximately 30 to 40% as potent as
epoprostenol.

Treprostinil and iloprost can also be administered by continuous

intravenous infusion; stability at room temperature and longer 4.5-hour half-
life are advantages over continuous intravenous epoprostenol. Treprostinil
administered by inhalation (four inhalations per day) is in clinical
development.

Endothelin Receptor Antagonists

Endothelin-1 (ET-1), one of the most potent vasoconstrictors

identified to date, has been implicated in the pathobiology of pulmonary
arterial hypertension. The ETA/ETS antagonist bosentan was the first
approved oral therapy for pulmonary arterial hypertension.

Bosentan is initiated at 62.5 mg twice daily for 4 weeks, then

increased to 125 mg twice daily. The orally active, selective ETA receptor
antagonists sitaxsentan (100 mg once daily) and ambrisentan (5 or 10 mg
once daily) are also approved. Adverse effects of endothelin receptor
antagonists include acute liver toxicity with an increase in hepatic
aminotransferase levels. Sitaxsentan also reduces the metabolism of warfarin.
Nitric Oxide and Phosphodiesterase Inhibitors

In pulmonary vascular smooth muscle cells, nitric oxide activates

guanylate cyclase, which increases cyclic guanosine monophosphate (cGMP)
and decreases intracellular calcium concentrations, thereby leading to
smooth muscle relaxation. When inhaled, the rapid combination of nitric
oxide with hemoglobin inactivates any nitric oxide diffusing into the blood,
thereby preventing systemic vasodilation. Nitric oxide is therefore a potent
and selective pulmonary vasodilator when administered by inhalation, thus
making it a useful short- term treatment of pulmonary hypertension; chronic
administration of nitric oxide, however, is cumbersome.

The pulmonary vasodilator effects of nitric oxide are mediated

through its second messenger, cGMP, which is rapidly degraded by
phosphodiesterase's. By selectively inhibiting phosphodiesterase type 5,
sildenafil promotes the accumulation of intracellular cGMP and enhances
nitric oxide - mediated vasodilation; it may also induce antiproliferative
effects in the pulmonary vasculature. Sildenafil improves exercise endurance,
hemodynamics, and functional class in patients with class II, III, or IV
pulmonary arterial hypertension.

The recommended starting dose is 20 mg orally three times a day, with an

increase to 40 or 80 mg orally three times daily if needed to achieve or
maintain its efficacy. More recently, clinical development continues with the
phosphodiesterase type 5 inhibitor tadalafil.

Combination Therapy Combining pharmacologic agents with different

mechanisms of action may produce an additive effect as under:
Mode of administration Starting dose Maximal
dose

Diltiazem PO 240–720 mg/day 960

mg/day

Nifedipine PO 90–180 mg/day 240

mg/day

Epoprostenol IV 2–4 ng/kg/min 25–40

ng/kg/min

Treprostinil IV/Sc 1–2 ng/kg/min 75–150

ng/kg/min

Beraprost Oral 20–100 m BID to QID

Iloprost Nebulizer system 6–9 times/day (2.5–5

mg/inhalation)

Bosentan Oral 62.5 mg BID (1–2 mg/kg twice daily) increased to 2–4
mg/kg after 4 weeks 125 mg BID

Sildenafil Oral 20 mg TID (1–5 mg/kg in 4 divided dosages) 80 mg TID

Tadalafil Oral 10 mg OD 40 mg OD

Selection of an agent: In general, the usage of the agents (when available)

can be guided by the following suggestion:

WHO functional class II: Preferred agents include ambrisentan, Bosentan,

Sildenafil, or Tadalafil.
WHO functional class III: Preferred agents include ambrisentan, Bosentan,
intravenous Epoprostenol, intravenous or subcutaneous Treprostinil, inhaled
Iloprost, Sildenafil, or Tadalafil.

WHO functional class IV: Patients with severe PH who are WHO
functional class IV should be treated with an intravenous prostanoid.

Most clinicians consider intravenous Epoprostenol to be the preferred agent.

Intravenous Treprostinil is considered a reasonable alternative by some.
Inhaled Iloprost can be considered for patients who refuse or cannot receive
intravenous therapy. Combination therapy is appropriate in cases refractory
to monotherapy. It should consist of two agents with different mechanisms
of action. In other words, it should consist of agents from any two of the
following three classes: prostanoids, endothelin receptor antagonists, and
PDE5 inhibitors.

SURGICAL MANAGEMENT OR INTERVENTIONAL THERAPY:

Right-to-Left Shunt:

Creation of a right-to-left shunt may be considered in adults with severe

symptomatic PH. These include atrial septostomy and Potts shunt.

The purpose of right-to-left shunting is to divert blood flow to bypass the

pulmonary vascular bed and enter the systemic circulation, thereby elevating
systemic blood flow and maintaining tissue perfusion, albeit with less
oxygenated blood.

Atrial septostomy

It is a surgical procedure in which a small hole is created between the

upper two chambers of the heart, the atria. This procedure is primarily used
to palliate dextro-Transposition of the great arteries or d-TGA (often
imprecisely called transposition of the great arteries), a life-threatening
cyanotic congenital heart defect seen in infants.

There are two types of this procedure: balloon atrial septostomy (also called
endovascular atrial septostomy, Rash kind a trial balloon septostomy, or
simply Rash kind's procedure) and blade atrial septostomy (also called static
balloon atrial septostomy

The majority of atrial septostomies are performed on infants with d-TGA or

other cyanotic heart defects. In these cases, a balloon catheter is guided
through a large vein into the right atrium, during cardiac catheterization. The
catheter is threaded into the foramen ovale, a naturally existing hole between
the atria that normally closes shortly after birth. The balloon at the end of the
catheter is inflated so as to enlarge the foramen ovule enough that it will no
longer become sealed. This allows more oxygenated blood to enter the right
heart (especially in the case of d-TGA) where it can be pumped to the rest of
the body. The balloon is deflated and the catheter is removed.

Sometimes the initial surgery is not entirely successful, or there are other
factors that make a simple balloon atrial septostomy impossible, such as an
older patient whose foramen ovule has already closed. This is when a blade
atrial septostomy is performed. The details of the procedure are largely the
same, except that a small blade on the end of the catheter is first used to
create an opening between the right and left atria, before the insertion of the
balloon.

The Rash kind balloon atrial septostomy is performed during cardiac

catheterization (heart Cath), in which a balloon catheter is used to enlarge a
foramen ovale, patent foramen ovale (PFO), or atrial septal defect (ASD) in
order to increase oxygen saturation in patients with cyanotic congenital heart
defects (CHDs).

Pulmonary thromboendarterectomy
It is an operation that removes organized clotted blood (thrombus) from the
pulmonary arteries, which supply blood to the lungs.

indication
Surgery is indicated in patients with pulmonary artery emboli that are
surgically accessible. Thrombi are usually the cause of recurrent/chronic
pulmonary emboli and therefore, of chronic thromboembolic pulmonary
hypertension (CTEPH).

PTEs involve a full cardiopulmonary bypass (CPB), deep hypothermia and

cardioplegia (a crystalline fluid which stops the heart from beating). Actual
removal of the embolus is carried out in a standstill operation (deep
hypothermia and periods of cessation of circulation). CPB is needed to
divert blood from the heart and lungs and supply the body with oxygen and
blood while the pulmonary vasculature is operated on. Cardioplegia is
initiated as the approach to the pulmonary arteries is performed through the
pericardium, a fibrous sac surrounding the heart. Furthermore, movement
from the heart makes delicate work on the closely attached pulmonary
arteries complex. Hypothermia is necessary as the embolus is very delicate
and the risk of disruption is high, in order to appropriately visualize the clot
and remove it a bloodless field is required. Clot visualization is achieved
through dissection of the pulmonary arteries which is technically challenging.
If possible, the clot is removed in a single piece to avoid the formation of
mobile emboli. In order to achieve this CPB is periodically stopped,
resulting in a complete cessation of blood circulation. This is only feasible if
the patient is hypothermic (cooled to 18-20C) as metabolism is slowed and
the body can better tolerate the resulting lack of blood supply. Circulatory
arrest is limited to 20-minute intervals to protect brain function. After each
interval of arrest circulation is continued for 10 minutes or until pulmonary
venous oxygen saturation is at least 90%. Bypass time is typically 345
minutes.

Lung transplantation, or pulmonary transplantation,

It is a surgical procedure in which a patient's diseased lungs are partially or

totally replaced by lungs which come from a donor. Donor lungs can be
retrieved from a living donor or a deceased donor. A living donor can only
donate one lung lobe.

Lung transplantation is the therapeutic measure of last resort for patients

with end-stage lung disease who have exhausted all other available
treatments without improvement. A variety of conditions may make such
surgery necessary. As of 2005, the most common reasons for lung
transplantation were:

 27% chronic obstructive pulmonary disease (COPD), including

emphysema;
 16% idiopathic pulmonary fibrosis;
 14% cystic fibrosis;
 12% idiopathic (formerly known as "primary") pulmonary
hypertension;
 5% alpha 1-antitrypsin deficiency;
 2% replacing previously transplanted lungs that have since failed;
  24% other causes, including bronchiectasis and sarcoidosis.

Contraindications:
Despite the severity of a patient's respiratory condition, certain pre-existing
conditions may make a person a poor candidate for lung transplantation:

 Concurrent chronic illness (e.g., congestive heart failure, kidney

disease, liver disease)
 Current infections, including HIV and hepatitis
o However, more and more often, hepatitis C patients are both
being transplanted and are also being used as donors if the
recipient is hepatitis C positive. Similarly, select HIV-infected
individuals have received lung transplants after being evaluated
on a case-by-case basis.
 Current or recent cancer
 Current use of alcohol, tobacco or illegal drugs
 Age
 Psychiatric conditions
 History of noncompliance with medical instructions

Types of lung transplant

Lobe
A lobe transplant is a surgery in which part of a living or deceased donor's
lung is removed and used to replace the recipient's diseased lung. In living
donation, this procedure requires the donation of lobes from two different
people, replacing a lung on each side of the recipient.
Single-lung
Many patients can be helped by the transplantation of a single healthy lung.
The donated lung typically comes from a donor who has been pronounced
brain-dead.

Double-lung
Certain patients may require both lungs to be replaced. This is especially the
case for people with cystic fibrosis, due to the bacterial colonization
commonly found within such patients' lungs; if only one lung were
transplanted, bacteria in the native lung could potentially infect the newly
transplanted organ.

Heart–lung
Some respiratory patients may also have severe cardiac disease which would
necessitate a heart transplant. These patients can be treated by a surgery in
which both lungs and the heart are replaced by organs from a donor or
donors.

A particularly involved example of this has been termed a "domino

transplant" in the media. First performed in 1987, this type of transplant
typically involves the transplantation of a heart and lungs into recipient A,
whose own healthy heart is removed and transplanted into recipient B.

Complementary and Alternative Therapies

nutrition

While none of these supplements specifically treats pulmonary hypertension,

they may help heart health and function.

 Coenzyme Q10 (CoQ10). Good for heart health, and may help lower
blood pressure. DO NOT take CoQ10 if blood thinners, such as
 warfarin (Coumadin) or clopidogrel (Plavix) are taken. CoQ10 may
increase clotting and make these medications less effective.
 L-carnitine. Improves endurance and is good for heart health. DO
NOT take L-carnitine blood thinners or thyroid hormone are
prescribed.
 Magnesium. Helps heart work better and may help lower blood
pressure. Magnesium interacts with many medications, as well as
other herbs and supplements.
 Potassium. Helps heart muscle contract. If diuretic is prescribed,
doctor may advice to take a potassium supplement. however. If
another kind of diuretic, taking a potassium supplement could be
dangerous.
 Vitamin E and vitamin C. Antioxidants that protect the heart and
strengthen the immune system. DO NOT take vitamin E if blood
thinners are advised.
 Taurine. Good for heart health and may help lower blood pressure.
Taurine may act like a diuretic,

Herbs

Herbs are a way to strengthen and tone the body's systems. Herbs are
available as dried extracts (capsules, powders, and teas), glycerites (glycerin
extracts), or tinctures (alcohol extracts). Unless otherwise indicated, make
teas with 1 tsp. herb per cup of hot water. Steep covered 5 to 10 minutes for
leaf or flowers, and 10 to 20 minutes for roots. Drink 2 to 4 cups per day.
You may use tinctures alone or in combination as noted.

These herbs have not been studied specifically for pulmonary hypertension.
  Hawthorn (Crataegus monogyna). Hawthorn has been shown to help
reduce symptoms of heart failure. Some people think it may help
lower blood pressure, although so far studies are lacking. Hawthorn
interacts with many drugs taken to treat heart disease, high blood
pressure, and heart failure.
 Linden (Tilia cordata). May help the body get rid of excess fluid.
Strain and cool. DO NOT take linden if you take diuretics (water pills)
or lithium.
 Garlic (Allium sativum). May help lower blood pressure. Garlic may
increase the risk of bleeding, especially if blood thinners, such as
warfarin (Coumadin) are taken. Garlic may also interact with other
medications.
 Rosemary (Rosmarinus officinalis). May help the body get rid of
excess fluid. DO NOT take rosemary if diuretics (water pills) are
taken.
 Dans hen (Salvia miltiorrhiza). Dilates blood vessels. DO NOT take
dans hen if you take blood thinners (anticoagulants). Dans hen may
also interact with other drugs, including clarithromycin (Biaxin),
cyclosporine (Neural, Sand immune), digoxin (Lanoxin), diltiazem
(Cardizem), lovastatin (Mevacor), birth control pills, and many others.

Physical Medicine

Castor oil pack. Apply oil to a clean, soft cloth, place on chest and cover
with plastic wrap. Place a heat source over the pack and let sit for 30 to 60
minutes. Use for 3 consecutive days, take 1 to 2 days off, and then repeat 3-
day cycle.
Contrast hydrotherapy. Alternate hot and cold applications to the chest.
Alternate 3 minutes hot with 1 minute cold. Repeat 3 times to complete 1 set.
Do 2 to 3 sets per day.

Steams. Using 3 to 6 drops of essential oils in a humidifier, vaporizer,

atomizer, or warm bath may help reduce shortness of breath and improve
circulation. Consider eucalyptus, rosemary, thyme, or lavender.

Acupuncture

Acupuncture may help improve circulation

NURSING MANAGEMENT:

NURSING ASSESSMENT:
In the history collection includes the:

Biographic data: occupation and living atmosphere are given priority.

Present medical history: each and every symptom with the characteristics are
cleared assessed.

Patients should be asked to describe their pain in terms of the following

characteristics: location, radiation, mode of onset, character, temporal
pattern, exacerbating and relieving factors, and intensity.

Describe dyspnea whether it Is all the time or presents with walking or doing
daily activities. aggravating and relieving factors.

Past history: any recent pulmonary or heart disease conditions, thyroid, liver
diseases. Diabetes, hypertension and any other systemic disease conditions.
Family history of pulmonary hypertension and usage of medications should
be assessed
Life style and personal history: information regarding alcohol, smoking
habits, exercise pattern, food preference, amount of daily fat intake and
water intake, occupation, sleeping pattern should be collected.

PHYSICAL EXAMINATION:

During the physical exam,

♦ Listen for abnormal heart sounds such as a loud pulmonic valve

sound, a systolic murmur of tricuspid regurgitation, or a gallop due to
ventricular failure.
♦ Examine the jugular vein in the neck for engorgement (jugular vein
distension)
♦ Examine the abdomen, legs, and ankles for fluid retention(edema).
♦ Examine nail beds for bluish tint indicates cyanosis.

NURSING DIAGNOSIS:

 Risk of decreased cardiac put related to increased vascular resistance

or myocardial ischemia as evidenced as cyanosis.
 Activity intolerance related to imbalance between oxygen supply and
demand as evidenced by dyspnea.
 Acute pain related to vasoconstriction and increased pressure in the
pulmonary arteries as manifested by patient's verbalization.
 Ineffective coping related to multiple changes in lifestyle as evidenced
by verbalization of inability to cope and ask for help.
 Fluid volume excess related to increased metabolic acidosis as
evidenced by edema.
 Knowledge deficit related lack of knowledge as evidenced by frequent
questioning regarding disease condition and treatment regimen.
NURSING PLANNING:

Risk of decreased cardiac put related to increased vascular resistance or

myocardial ischemia as evidenced as cyanosis.

Nursing Interventions Rationale

Nursing Assessment
Monitor and record BP. Comparison of pressures provides a more
Measure in both arms and complete picture of vascular involvement or
thighs three times, 3–5 min scope of problem.
apart while patient is at rest,
then sitting,
Note presence, quality of Bounding carotid, jugular, radial, and femoral
central and peripheral pulses. pulses may be observed and palpated. Pulses in
the legs and feet may be diminished, reflecting
effects of vasoconstriction (increased systemic
vascular resistance [SVR]) and venous
congestion.
Auscultate heart tones and S4 heart sound is common in severely
breath sounds. hypertensive patients because of the presence of
atrial hypertrophy (increased atrial volume and
pressure). Development of S3 indicates
ventricular hypertrophy and impaired
functioning. Presence of crackles, wheezes may
indicate pulmonary congestion secondary to
developing or chronic heart failure.
Observe skin colour, moisture, Presence of pallor; cool, moist skin; and delayed
temperature, and capillary capillary refill time may be due to peripheral
refill time. vasoconstriction or reflect cardiac
decompensation and decreased output.
Note dependent and general May indicate heart failure, renal or vascular
oedema. impairment.
Evaluate the evidence of To assess for signs of poor ventricular function
extreme fatigue, intolerance or impending cardiac failure.
for activity, sudden or
progressive weight gain,
swelling of extremities, and
progressive shortness of
breath.
Therapeutic Interventions
Provide calm, restful Helps lessen sympathetic stimulation; promotes
surroundings, minimize relaxation.
environmental activity and
noise. Limit the number of
visitors and length of stay.
Maintain activity restrictions Lessens physical stress and tension that affect
(bedrest or chair rest); blood pressure and the course of hypertension.
schedule periods of
uninterrupted rest; assist
patient with self-care activities
as needed.
Provide comfort measures Decreases discomfort and may reduce
(back and neck massage, sympathetic stimulation.
elevation of head).
Instruct in relaxation Can reduce stressful stimuli, produce calming
techniques, guided imagery, effect, thereby reducing BP.
distractions.
Administer medication as per To reduce the sign and symptoms.
doctors' advice
dietary sodium, fat, and These restrictions can help manage fluid
cholesterol restrictions as retention and, with associated hypertensive
indicated. response, decrease myocardial workload.

Activity intolerance related to imbalance between oxygen supply and

demand as evidenced by dyspnea.
Evaluate client’s actual and
perceived limitations or degree of
deficit in light of usual status.
Assess the patient’s response to
activity.
Assess emotional and
psychological factors affecting
the current situation.
Therapeutic Interventions
Instruct patient in energy-
conserving techniques
Encourage progressive activity
and self-care when tolerated.
Provide assistance as needed.
Fluid volume excess related to increased metabolic acidosis as evidenced by
edema.
instruct patient, caregiver, and
family members regarding fluid
restrictions, as appropriate.
Limit sodium intake as prescribed.
Monitor fluid intake.
Administer diuretics as prescribed.
Elevate oedematous extremities,
and handle with care.
Provides comparative baseline and provides
information about needed education and
interventions regarding quality of life.
helpful in assessing physiological responses
to the stress of activity and, if present, are
indicators of overexertion.
Stress or depression may be increasing the
effects of an illness, or depression might be
the result of being forced into inactivity.
Energy-saving techniques reduce the energy
expenditure, thereby assisting in equalization
of oxygen supply and demand.
Gradual activity progression prevents a
sudden increase in cardiac workload.
Providing assistance only as needed
encourages independence in performing
activities.
Information and knowledge about condition
are vital to patients who will be co-
managing fluids.
Restriction of sodium aids in decreasing
fluid retention
This enhances compliance with the
regimen.
Diuretics aids in the excretion of excess
body fluids.
Elevation increases venous return to the
heart and, in turn, decreases oedema.
Oedematous skin is more susceptible to
injury.
Place the patient in a semi-
Fowler’s or high-Fowler’s
position.
Aid with repositioning every 2
hours if the patient is not mobile.
Explain the need to use ant
embolic stockings or bandages, as
ordered.
Raising the head of bed provides comfort in
breathing.
Repositioning prevents fluid accumulation
in dependent areas.
These aids help promote venous return and
minimize fluid accumulation in the
extremities.

COMPLICATIONS:

 Right-sided heart enlargement and heart failure (cor pulmonale).

In cor pulmonale, heart's right ventricle becomes enlarged and has to pump
harder than usual to move blood through narrowed or blocked pulmonary
arteries.

At first, the heart tries to compensate by thickening its walls and expanding
the chamber of the right ventricle to increase the amount of blood it can hold.
But this thickening and enlarging works only temporarily, and eventually the
right ventricle fails from the extra strain.

 Blood clots.

Clots help stop bleeding after injury. But sometimes clots form where
they're not needed. A number of small clots or just a few large ones dislodge
from these veins and travel to the lungs, leading to a form of pulmonary
hypertension that can generally be reversible with time and treatment.

Having pulmonary hypertension makes it more likely develop clots in the

small arteries in your lungs, which is dangerous if already they have
narrowed or blocked blood vessels.
  Arrhythmia.

Irregular heartbeats (arrhythmias) from the upper or lower chambers of the

heart are complications of pulmonary hypertension. These can lead to
palpitations, dizziness or fainting and can be fatal.

 Bleeding.

Pulmonary hypertension can lead to bleeding into the lungs and coughing
up blood (hemoptysis). This is another potentially fatal complication.

PREVENTION:

PRIMAY PREVENTION:

 Maintaining a Healthy Weight Carrying any excess weight in body

places extra workload on heart.

Regular Exercise Maintaining a physically active lifestyle can not only

deliver benefits such as reducing blood pressure, but it can also reduce
ongoing risk of a cardiac event. It is recommended that complete around
30mins most days, if not all days, of moderate physical activity.
 Maintaining a healthy diet is not difficult, it means making simple
and healthy food choices and should aim to eat a diet rich in
vegetables, wholegrains, lean meats, fruit and low-fat dairy; limiting
high saturated fat foods as well as reducing salt levels is also
recommended. Furthermore, increasing the consumption of foods
such as legumes and complex carbohydrates could be beneficial, as
well as limiting alcohol to 2x standard drinks per day.
 Cessation of Smoking is one of the leading risk factors for cardiac
events.
  Managing Stress Some useful ways to manage stress in daily life
could be to try techniques and exercises such as mediation, breathing
techniques, visualizations and muscle relaxation.
 Maintain a Healthy Blood Pressure aim for an optimal blood pressure
of less than 140/90mmHg, as anything from that range and above
increases risk of Heart Disease. High blood pressure, also known as
Hypertension, can be caused by excess salt, being overweight, high
alcohol levels, smoking as well as family history.
SECONDARY PREVENTION:

 Avoid pregnancy
 Rigorous follow up in case of pregnancy
 Avoid unnecessary surgeries
 Multidisciplinary care in case of necessary surgery
 Avoid high altitude
 Supplemental oxygen in order to ensure a target oxygen saturation of
91% in case of exposure to high altitude
 Up-to-date immunizations against influenza and pneumococcal
pneumonia

TERTIARY PREVENTION

Pulmonary Rehabilitation includes:

 Exercise training
 Education
 Nutritional therapy
 Psychosocial / Behavioral intervention
 Outcome assessment
  Promotion of long-term adherence

PROGNOSIS:

Pulmonary hypertension is considered a universally fatal illness, although

survival time may vary between individuals. The prognosis of pulmonary
arterial hypertension (WHO Group I) has an untreated median survival of 2–
3 years from time of diagnosis, with the cause of death usually being right
ventricular failure (cor pulmonale). A recent outcome study of those patients
who had started treatment with bosentan (Treaclier) showed that 89% of
patients were alive at 2 years.

Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension

Disease Management (REVEAL Registry) from March 2006 to December
2009, 1-, 3-, 5-, and 7-year survival rates were 85%, 68%, 57%, and 49%,
respectively. For patients with idiopathic/familial pulmonary hypertension,
survival rates were 91%, 74%, 65%, and 59%. Levels of mortality are very
high in pregnant women with severe pulmonary arterial hypertension (WHO
Group I).

FOLLOW UP:

As the pulmonary hypertension is fatal illness follow up is very important

for the prevention of worsening of condition and complication.

RESEARCH STUDY:

Metformin in Pulmonary Hypertension in Left Heart Disease

Vinaya Mulkareddy and Marc A. Simon
Metformin is ubiquitously used in the management of Type II Diabetes
Mellitus (DMII). Over the years, our growing knowledge of its therapeutic
potential has broadened its use to the treatment of infertility in polycystic
ovarian syndrome, gestational diabetes, and even obesity. Recently, it has
been suggested as a novel therapy in cardiovascular disease (CVD). Given
that CVD is the leading cause of death in patients with DMII, with ~ 75%
dying from a cardiovascular event, the intersection of DMII and CVD
provides a unique therapeutic target. In particular, pulmonary hypertension
(PH) related to CVD (Group II PH) may be an optimal target for metformin
therapy.

Clinical trials:

In a compelling pilot study recently completed, Mohan et al. assessed the

therapeutic benefit of metformin on LV hypertrophy in 68 patients with
coronary artery disease without diabetes. They showed metformin decreased
LV mass, systolic blood pressure, body weight, and oxidative stress as
measured by thiobarbituric acid reactive substances in patients with
preserved LVEF. Another trial in 62 non-diabetic insulin resistant HFrEF
patients found improvements in functional class and minute
ventilation/carbon dioxide production (VE/VCO2 slope). We are currently
pursuing a prospective phase II clinical trial evaluating the therapeutic
efficacy of metformin in PH-HFpEF which will evaluate exercise
hemodynamics, functional capacity, skeletal muscle signaling, and insulin
sensitivity.

Metformin in pulmonary hypertension and left heart disease

he therapeutic role of metformin is also being investigated in both

pulmonary arterial hypertension (PAH) and HFpEF. There is a growing
body of evidence that PAH is associated with a number of systemic
metabolic derangements including metabolic syndrome, insulin resistance,
and glucose intolerance. Alterations in aerobic glycolysis, tricarboxylic acid
metabolism, and fatty oxidation have all been implicated in the
pathophysiology. Given these findings, there is at least one currently
ongoing clinical trial looking at the role of metformin in PAH. While in
HFpEF there is significant clinical heterogeneity, there is increasing
evidence illustrating metformin's therapeutic benefit across the varying
phenotypes. Halabi et al. recently published a systematic review and meta
regression analysis analyzing the role of metformin in HFpEF. Metformin
reduced mortality and morbidity in HFpEF patients even after adjustment for
other HF and glycemic control therapies including beta blockers, angiotensin
converting enzyme inhibitors, and insulin.

SUMMARY:

Pulmonary hypertension (PH or PHTN) is a condition of increased blood

pressure within the arteries of the lungs. Symptoms include shortness of
breath, syncope, tiredness, chest pain, swelling of the legs, and a fast
heartbeat. The condition may make it difficult to exercise. Onset is typically
gradual. if the pulmonary mean arterial pressure is greater than 25mmHg at
rest, or greater than 30mmHg during exercise. While the exact frequency of
the condition is unknown, it is estimated that about 1,000 new cases occur a
year in the United States. Females are more often affected than males. Onset
is typically between 20 and 60 years of age. It was first identified by Ernst
von Romberg in 1891.

The cause is often unknown. Risk factors include a family history, prior
blood clots in the lungs, HIV/AIDS, sickle cell disease, cocaine use, chronic
obstructive pulmonary disease, sleep apnea, living at high altitudes, and
problems with the mitral valve. The underlying mechanism typically
involves inflammation and subsequent remodeling of the arteries in the
lungs. Diagnosis involves first ruling out other potential causes.

The pathogenesis of pulmonary arterial hypertension (WHO Group I)

involves the narrowing of blood vessels connected to and within the lungs.
This makes it harder for the heart to pump blood through the lungs, as it is
much harder to make water flow through a narrow pipe as opposed to a wide
one. Over time, the affected blood vessels become stiffer and thicker, in a
process known as fibrosis. The mechanisms involved in this narrowing
process include vasoconstriction, thrombosis, and vascular remodeling
(excessive cellular proliferation, fibrosis, and reduced
apoptosis/programmed cell death in the vessel walls, caused by
inflammation, disordered metabolism and dysregulation of certain growth
factors). This further increases the blood pressure within the lungs and
impairs their blood flow. In common with other types of pulmonary
hypertension, these changes result in an increased workload for the right side
of the heart. The right ventricle is normally part of a low-pressure system,
with systolic ventricular pressures that are lower than those that the left
ventricle normally encounters. As such, the right ventricle cannot cope as
well with higher pressures, and although right ventricular adaptations
(hypertrophy and increased contractility of the heart muscle) initially help to
preserve stroke volume, ultimately these compensatory mechanisms are
insufficient; the right ventricular muscle cannot get enough oxygen to meet
its needs and right heart failure follows. As the blood flowing through the
lungs decreases, the left side of the heart receives less blood. This blood may
also carry less oxygen than normal. Therefore, it becomes harder and harder
for the left side of the heart to pump to supply sufficient oxygen to the rest
of the body, especially during physical activity.

There is currently no cure for pulmonary hypertension, although research on

a cure is ongoing. Treatment depends on the type of disease. A number of
supportive measures such as oxygen therapy, diuretics, and medications to
inhibit blood clotting may be used. Medications specifically used to treat
pulmonary hypertension include epoprostenol, treprostinil, iloprost,
bosentan, ambrisentan, macitentan, and sildenafil. Lung transplantation may
be an option in severe cases. A 1973 World Health Organization meeting
was the first attempt to classify pulmonary hypertension by its cause, and a
distinction was made between primary PH (resulting from a disease of the
pulmonary arteries) and secondary PH (resulting secondary to other, non-
vascular causes). Further, primary PH was divided into the "arterial
plexiform", "veno-occlusive" and "thromboembolic" forms. World
Symposia on PAH have further defined the classification of PH. The
classification continues to evolve based on improved understanding of the
disease mechanisms. PAH is considered a universally fatal illness, although
survival time may vary between individuals. The prognosis of pulmonary
arterial hypertension (WHO Group I) has an untreated median survival of 2–
3 years from time of diagnosis, with the cause of death usually being right
ventricular failure (cor pulmonale).

CONCLUSION:

As pulmonary hypertension is fatal illness as nurses, we need to know

in detail about the etiology, management and its prevention for better care of
patients.
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