Cor Pulmonale and Pulmonary Hypertension

Cor pulmonale is right heart failure that results from increased pulmonary pressure. Cor pulmonale is sometimes also called right ventricular hypertrophy, a condition in which the right ventricle is damaged and blood flow is interrupted. The right ventricle is a pumping chamber in the lower part of the heart responsible for exporting oxygen poor blood to the lungs for oxygen renewal. Cor pulmonale may result from pulmonary hypertension can develop as follows:

There are thousands of capillaries in the lung that export oxygen rich blood to pulmonary arteries and import oxygen depleted blood for oxygen renewal. The interior of these capillaries are lined with endothelial cells (cells that prevent blood from leaking out). The outer walls are made up of a muscle type cells that expand and contract to propel blood through the blood vessel. When primary pulmonary hypertension occurs, the endothelial cells are weakened so that they allow leakage. This leakage in turn causes the muscles type cells surrounding the blood vessels to constrict and inhibit blood flow. The constriction gradually worsens, increasing the pulmonary arterial pressure, eventually causing cor pulmonale.

The initial symptoms of pulmonary hypertension are generally common complaints. Patients typically experience only a general fatigue or tiredness and in most cases imagine that they are simply "out-of-shape." As the condition worsens and right ventricular hypertrophy develops, patients experience more acute symptoms such as difficulty in breathing, dizziness and even fainting spells. As time passes, more severe symptoms; swelling in the ankles or legs, bluish discoloration of the lips and skin, and chest pain also develop.

The median survival rate for Primary pulmonary hypertension after diagnosis is less than two and a half years. Cor pulmonale occurs in patients with advanced PPH and therefore, once it develops, the survival rate is shorter. Medical management of PPH as discussed extensively on this web site, consists of anticoagulants, oral calcium channel blocking vasodilators (which are effective only in 20%-25% of cases), continuous intravenous infusions of prostacyclin, diuretics, and supplemental oxygen.

Because of the subtle development of symptoms, early detection of PPH is unlikely and even when it is suspected, a series of multiple tests to either confirm or dismiss the possibility of this diagnosis are required.

No one is certain as to what causes this disease, but research has shown that there are a number of factors that might be responsible. Some of the possible causes include immune system disease, congenital heart defects, Collagen Vascular Disease, HIV infection, portal hypertension and intravenous drug use or other chemical exposures. The use of certain appetite suppressants as Pondimin (fenfluramine), Redux (dexfenfluramine) and Fen Phen, have been found to greatly increase the risk of developing PPH. An echocardiogram is a good tool for detecting elevated pulmonary artery pressures. However, with some patients, a cardiac catheterization is the only way to confirm the diagnosis.

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