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Primary pulmonary hypertension is a rare condition in which blood vessels in the lungs become narrowed. This causes high blood pressure in the arteries that supply the lungs (pulmonary arteries) and restricted blood flow to the lungs.

As the blood pressure rises in the lungs, the elevated pressure is transmitted back to the right ventricle of the heart. This can result in stretching of the tissue that supports the valve between the right atrium and right ventricle (tricuspid valve), causing the valve to leak.

Manifestations of primary pulmonary hypertension include fatigue and shortness of breath that become progressively worse with time. Other signs and symptoms include angina (chest pain), blackouts (syncope) and blueness of skin (cyanosis). In later stages, symptoms such as swelling in the legs, enlargement and pain in the abdomen, loss of appetite and bulging of the jugular veins in the neck may occur.

In most cases, the cause of the pulmonary hypertension can’t be determined and it is thus termed primary (of undetermined cause). Use of some diet medications including fenfluramine (the ‘fen’ in the fen-phen combination) and dexfenfluramine (Redux) has been associated with the development of pulmonary hypertension. These drugs also have been implicated in valvular heart disease, which is often characterized by leakage of the heart valves. Some people who have used these diet drugs may develop both pulmonary hypertension and valvular heart disease.