Primary Pulmonary Hypertension Symptoms Treatment
Definition of Pulmonary Hypertension:
Pulmonary hypertensionis blood vessel disorder of the lung artries
in which the pressure in the pulmonary artery (the
blood vessel that leads from the heart to the
lungs) rises above normal levels and may become life threatening. The blood pressure measured by cuff on your arm isn't
directly related to the pressure in your lungs. The blood vessels that supply the lungs constrict
and their walls thicken, so they can't carry as much blood. The heart works harder, trying to force
the blood through. If the pressure is high enough, eventually the heart can't
keep up, and less blood can circulate through the lungs to pick
up oxygen.Normal blood pressure of pulmonary arteries is
In some people, the bone marrow produces more red blood cells to compensate for less oxygen in
the blood, leading to a condition called
polycythemia . The extra red blood cells cause the blood to become thicker and stickier, further
increasing the load on the heart. These changes also put a person with cor pulmonale at
increased risk of pulmonary embolism because the thickened blood may clump and form clots, mainly in the
veins of the legs. These clots can dislodge and travel to the lungs.
Types and causes of of Pulmonary Hypertension:
Primary PH: Cause not known. PPH is extremely rare, occurring in about two persons per million
population per year.
Secondary PH: Cause known. Secondary to lungs eg asthama, emphysima, Cor pulmonale,
inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus
erythematosus (SLE). Congenital heart diseases and heart diseases eg Left heart failure..Congenital
heart diseases that cause shunting of extra blood through the lungs like ventricular and
atrial septal defects, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery), HIV
infection, liver disease and diet drugs like fenfluramine and dexfenfluramine are also causes
of pulmonary hypertension.
Symptoms of Pulmonary
Shortness of breath upon exertion is the most common symptom of pulmonary hypertension, and
virtually everyone who has the condition develops it. Some people feel light-headed or fatigued
upon exertion, and an angina-like chest pain is common. The person is likely to feel weak because
body tissues are not receiving enough oxygen. Other symptoms, such as coughing and wheezing, are
usually caused by the underlying lung disease. Swelling (edema), particularly of the legs, may
occur because fluid may leak out of the veins and into the tissues, but this is usually a sign that
cor pulmonale has developed.
Diagnosis of Pulmonary
History and a careful physical examination are paramount to the diagnosis of pulmonary
hypertension. Particular attention should be given to previous medical conditions, drug use (legal
and illegal) and family history. In addition, all systems should be carefully reviewed. Commonly,
suspicion is increased by the presence of increasing dyspnea on exertion in a patient with a known
cause of pulmonary hypertension.
Electrocardiogram demonstrating the changes of right ventricular hypertrophy (long arrow) with
strain in a patient with primary pulmonary hypertension and Right axis
Chest Xray shows right ventricular hypertrophy.
Patient should undergo two-dimensional echocardiography with Doppler flow studies.
Echocardiography is the most useful imaging modality for detecting pulmonary hypertension and
excluding underlying cardiac disease. Confirmation of pulmonary hypertension is based on
identification of tricuspid regurgitation. The addition of mean right atrial pressure to the peak tricuspid jet velocity
gives an accurate noninvasive estimate of peak pulmonary pressure. Right ventricular dilatation and
hypertrophy are late findings.
Arterial blood gas analysis should be performed to
exclude hypoxia and acidosis as contributors to pulmonary hypertension.
Pulmonary function tests are necessary to establish airflow obstruction or restrictive
pulmonary pathology. Computed tomographic (CT) scanning of the chest with high-resolution images is useful for excluding
occult interstitial lung disease and mediastinal fibrosis when the pulmonary function tests
and chest radiograph are nondiagnostic.
Cardiac catheterization should be performed in patients with unexplained pulmonary hypertension,
and remains the gold standard for its diagnosis and quantification. Catheterization is particularly
useful in diagnosing occult shunts, congenital heart disease and distal pulmonary artery
Treatment of Pulmonary
Treatment goals are
dilate pulmonary vessels by calcium channel blockers, prostacyclins Nitric oxide
Decrease thickening of blood by warferin
Decrease preload on heart by diuretics and decrease salt intake
Increase oxygen supply to lungs
Prevention and treatment of respiratory infection by antibiotics
Increase cardiac output by inotropic drugs
Treat cause of secondary Pulmonary hypertension.
Calcium channel blockers may alleviate pulmonary vasoconstriction and prolong life in
about 20 percent of patients with primary pulmonary hypertension.
Epoprostenol (Flolan), or prostacyclin, is the single most important advance in the
treatment of primary pulmonary hypertension. This potent, short-acting vasodilator and
inhibitor of platelet aggregation is produced by vascular endothelium. In one
study,continuous intravenous infusion of epoprostenol improved exercise capacity,
quality of life, hemodynamics and long-term survival in patients with class III or IV
functionAlthough the delivery system for continuous infusion is complex, most patients
are able to learn how to prepare and infuse the drug.
Chronic anticoagulation with warfarin (Marivan) is recommended to prevent
thrombosis and has been shown to prolong life in patients with primary pulmonary
hypertension.1 Patients with this condition are prone to thromboembolism because of
sluggish pulmonary blood flow, dilated right heart
chambers, venous insufficiency and relative physical inactivity. Maintaining
an INR of 1.5 to 2.0 is recommended.
Because hypoxia is a potent pulmonary vasoconstrictor, it is critical to identify
and reverse hypoxemia. Low-flow supplemental oxygen therapy prolongs survival in
hypoxemic patients. Failure to recognize and correct hypoxemia may be the error most
frequently made in the treatment of patients with pulmonary hypertension. Pt should
have oxygen cylinder with them at high altitudes.
A low-salt diet and judicious use of diuretics can be helpful in reducing volume
overload in patients with pulmonary hypertension and right ventricular failure. Because
the right heart is dependent on preload, care should be taken to avoid excessive
diuresis and further reduction of cardiac output.
In patients with secondary pulmonary hypertension, management is directed at early
recognition and treatment of the underlying disease (while it is still potentially
reversible). For instance, left ventricular dysfunction should be treated with
afterload-reducing agents, digoxin and diuretics. Surgery to correct structural cardiac
and pulmonary anomalies can also be effective, and thromboendarterectomy for accessible
chronic thromboemboli is potentially curative. Improvement or resolution of pulmonary
hypertension may occur after the discontinuation of anorectic agents, although
resolution is not typical. Pulmonary hypertension associated with interstitial lung
disease may respond to corticosteroids or other immunosuppressive agents.
Primary pulmonary hypertension is usually progressive and ultimately fatal. Lung
transplantation is an option in some patients younger than 65 years who have pulmonary
hypertension that does not respond to medical management. According to a 1997 U.S.
transplant registry report, lung transplant recipients with primary pulmonary
hypertension had survival rates of 73 percent at one year, 55 percent at three years
and 45 percent at five years. The immediate reduction in pulmonary artery
pressure is associated with an
improvement in right ventricular function. Recurrence of primary pulmonary
hypertension after lung transplantation has not been reported.
Prognosis of Pulmonary
The median duration of survival after the diagnosis of primary pulmonary hypertension is 2.8
years, but this figure is highly variable. As a result of new treatments,
patients without hemodynamic evidence of right ventricular dysfunction may survive for more
than 10 years. The prognosis for patients with secondary pulmonary hypertension depends on
the underlying disease, as well as right ventricular function. For instance, patients with
COPD and moderate airflow obstruction have a three-year mortality rate of 50 percent after
the onset of right ventricular failure. Survival is similarly influenced in patients with
interstitial lung disease and pulmonary hypertension.