PULMONARY HYPERTENSION

INTRODUCTION

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. These pressures cannot be measured indirectly as
can systemic blood pressure; instead, they must be measured during right-sided
heart catheterization. In the absence of these measurements, clinical recognition
becomes the only indicator for the presence of pulmonary hypertension.

MEANING

pulmonary hypertension (PH) is an increase in blood pressure in

thepulmonary artery, pulmonary vein, or pulmonary capillaries, together known
as the lung vasculature, leading to shortness of breath, dizziness, fainting, and
other symptoms, all of which are exacerbated by exertion. Pulmonary
hypertension can be a severe disease with a markedly decreased exercise
tolerance and heart failure.

TYPES

World Health Organization was done the first attempt to classify

pulmonary hypertension. In 2003, the 3rd World Symposium on Pulmonary
Arterial Hypertension was convened in Venice to modify the classification
based on new understandings of disease mechanisms.

I) The Venice 2003 Revised Classification system

 WHO Group I - Pulmonary arterial hypertension (PAH)

 Idiopathic (IPAH)

 Familial (FPAH)

 Associated with other diseases (APAH): collagen vascular

disease (e.g. scleroderma), congenital shunts between the
systemic and pulmonary circulation, portal
 hypertension,HIV infection, drugs, toxins, or other diseases
or disorders

 Associated with venous or capillary disease

 WHO Group II - Pulmonary hypertension associated with left heart

disease

 Atrial or ventricular disease

 Valvular disease (e.g. mitral stenosis)

 WHO Group III - Pulmonary hypertension associated with lung

diseases and/or hypoxemia

 Chronic obstructive pulmonary

disease (COPD), interstitial lung disease (ILD)

 Sleep-disordered breathing, alveolar hypoventilation

 Chronic exposure to high altitude

 Developmental lung abnormalities

 WHO Group IV - Pulmonary hypertension due to chronic thrombotic

and/or embolic disease

 Pulmonary embolism in the proximal or distal

pulmonary arteries

 Embolization of other matter, such as tumor cells

or parasites

 WHO Group V - Miscellaneous

II) There are two forms of pulmonary hypertension: primary (or

idiopathic) and secondary. Primary pulmonary hypertension is an
uncommon disease in which the diagnosis is made by excluding all
other possible causes. The exact cause is unknown, but there are
several possible causes. The clinical presentation of primary
pulmonary hypertension exists with no evidence of pulmonary and
 cardiac disease or pulmonary embolism. It occurs most often in
women 20 to 40 years of age and is usually fatal within 5 years of
diagnosis.
III) According to the most recent classification, it can be one of five
different types: arterial, venous, hypoxic,
thromboembolic or miscellaneous.

CAUSES OF PULMONARY HYPERTENSION

A) Primary or Idiopathic
 Altered immune mechanisms
 Silent pulmonary emboli
 Raynaud’s phenomenon
 Oral contraceptive use
 Sickle cell disease
 Collagen diseases
B) Secondary

 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)

Secondary pulmonary hypertension is more common and results from

existing cardiac or pulmonary disease. The prognosis depends on the severity of
the underlying disorder and the changes in the pulmonary vascular bed. A
common cause of secondary pulmonary hypertension is pulmonary artery
constriction due to hypoxemia from COPD.

PATHOPHYSIOLOGY

The underlying process of pulmonary hypertension varies, and multiple

factors are often responsible.
Normally, the pulmonary vascular bed can handle the blood volume
delivered by the right ventricle.
It has a low resistance to blood flow and compensates for increased blood
volume by dilation of the vessels in the pulmonary circulation.
However, if the pulmonary vascular bed is destroyed or obstructed, as in
pulmonary hypertension, the ability to handle whatever flow or volume of
blood it receives is impaired, and the increased blood flow then increases
the pulmonary artery pressure.
As the pulmonary arterial pressure increases, the pulmonary vascular
resistance also increases.
Both pulmonary artery constriction (as in hypoxemia or hypercapnia) and
a reduction of the pulmonary vascular bed (which occurs with pulmonary
emboli) result in an increase in pulmonary vascular resistance and
pressure.
This increased workload affects right ventricular function.
The myocardium ultimately cannot meet the increasing demands imposed
on it, leading to right ventricular hypertrophy (enlargement and dilation)
and failure.

CLINICAL MANIFESTATIONS

 Dyspnea is the main symptom of pulmonary hypertension, occurring at

first with exertion and eventually at rest.
 Substernal chest pain also is common, affecting 25% to 50% of patients.
  Other signs and symptoms include
 weakness,
 fatigue,
 syncope,
 occasional hemoptysis,
  non-productive cough,
 and signs of right-sided heart failure (peripheral edema, ascites,
distended neck veins, liver engorgement, crackles, heart murmur).

ASSESSMENT AND DIAGNOSTIC FINDINGS

A complete diagnostic evaluation includes

 a history,
 physical examination,
These include altered heart sounds, such as a widely split S2 or second heart
sound, a loud P2or pulmonic valve closure sound (part of the second heart
sound), (para)sternal heave, possible S3 or third heart sound, and pulmonary
regurgitation. Other signs include an elevatedjugular venous
pressure, peripheral edema (swelling of the ankles and feet), ascites (abdominal
swelling due to the accumulation of fluid), hepatojugular reflux, and clubbing.

 chest x-ray,
 pulmonary function studies,
 electrocardiogram (ECG),
 Echocardiogram: An echocardiogram can assess the progression of the
disease and rule out other conditions with similar signs and symptoms.
 ventilation–perfusion scan, and
 cardiac catheterization.
 In some cases, a lung biopsy, performed by thoracotomy or
thoracoscopy, may be needed to make a definite diagnosis. Cardiac
catheterization of the right side of the heart reveals elevated pulmonary
arterial pressure. The ECG reveals right ventricular hypertrophy, right
axis deviation, and tall peaked P waves in inferior leads, tall anterior R
waves, and ST-segment depression and/or T-wave inversion anteriorly.
The PaO2 also is decreased (hypoxemia). A ventilation–perfusion scan
or pulmonary angiography detects defects in pulmonary vasculature,
such as pulmonary emboli. Pulmonary function studies may be normal or
 show a slight decrease in vital capacity (VC) and lung compliance, with
a mild decrease in the diffusing capacity.

MEDICAL MANAGEMENT

The goal of treatment is to manage the underlying cardiac or pulmonary

condition.
 Most patients with primary pulmonary hypertension do not have
hypoxemia at rest but require supplemental oxygen with exercise.
 Patients with severe right ventricular failure, decreased cardiac output,
and progressive disease may have resting hypoxemia and require
continuous oxygen supplementation. Appropriate oxygen therapy
reverses the vasoconstriction and reduces the pulmonary hypertension in
a relatively short time.
 In the presence of cor pulmonale, treatment should include fluid
restriction, diuretics to decrease fluid accumulation, cardiac glycosides
(eg, digitalis) in an attempt to improve cardiac function, calcium channel
blockers for vasodilation, and rest.
 In primary pulmonary hypertension, vasodilators have been administered
with variable success (eg, calcium channel blockers, intravenous
prostacyclin). Prostacyclin (PGX [Flolan]) is one of the prostaglandins
produced by the pulmonary endothelium.
 Intravenous prostacyclin (epoprostenol) helps to decrease pulmonary
hypertension by reducing pulmonary vascular resistance and pressures
and increasing cardiac output.
 Anticoagulants such as warfarin (Coumadin) have been given to patients
because of chronic pulmonary emboli.

SURGICAL MANAGEMENT
a) Atrial septostomy is a surgical procedure that creates a communication
between the right and left atria. It relieves pressure on the right side of the
heart, but at the cost of lower oxygen levels in blood (hypoxia).
b) Lung transplantation cures pulmonary arterial hypertension, but leaves
the patient with the complications of transplantation, and a post-
surgical median survival of just over five years.
 c) Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is
used for chronic thromboembolic pulmonary hypertension. It is the
surgical removal of an organized thrombus(clot) along with the lining of
the pulmonary artery; it is a very difficult, major procedure that is
currently performed in a few select centers. Case series show remarkable
success in most patients.
 Heart– lung transplantation has been successful in selected patients with
primary hypertension who have not been responsive to other therapies.

NURSING MANAGEMENT

 Activity intolerance related to impaired respiratory function

 Risk for deficient fluid volume related to fever and dyspnea
 Imbalanced nutrition: less than body requirements
 Deficient knowledge about the treatment regimen and preventive
health measures

 The major nursing goal is to identify patients at high risk for

pulmonary hypertension, such as those with COPD, pulmonary
emboli, congenital heart disease, and mitral valve disease.
 The nurse also must be alert for signs and symptoms, administer
oxygen therapy appropriately, and instruct patients and their
families about the use of home oxygen supplementation.

COMPLICATIONS

 Right-sided heart failure (cor pulmonale). In cor pulmonale, your

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 you've been injured. 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 is reversible with time and treatment.
Having pulmonary hypertension makes it more likely you'll develop clots in the
small arteries in your lungs, which is dangerous if you already 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.

REFERENCE

1. Suzanne.C.smeltzes, Brenda G Bare; “TEXT BOOK OF MEDICAL –

SURGICAL NURSING”, 10 th Edition, Lippincotl Williams & wilkins.
2. Joyce.M.Black, Jane Hokanson Hawks, “MEDICAL SURGICAL
NURSING”; 7th Edition, Elsevier publications.
3. Lewis, Heitkemper, Dirksen, O’ Brien, Buch ex; “MEDICAL –
SURGICAL NURSING”; 7th Edition, Mosby publications.
4. Phips Wilma J, Shafer’s, “MEDICAL – SURGICAL NURSING”; 7 th
Edition B.I. Publications”.
5. http://en.wikipedia.org/wiki/Pulmonary_hypertension
6. http://ezinearticles.com/Primary-Pulmonary-Hypertension-Complication
&id=144323
7. http://www.mayoclinic.com/health/pulmonary-hypertension/ DS00430 /
DSECTION=complications