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Kinds of pulmonary arterial hypertension and their causes:
Idiopathic pulmonary arterial hypertension has no known cause. Ten percent of idiopathic PAH cases are estimated to be inherited. Although it can strike children and men, the disease occurs most frequently among young women.
In secondary pulmonary arterial hypertension the cause is known and can include such breathing disorders as emphysema and bronchitis, inflammatory or collagen vascular diseases such as scleroderma, lupus or rheumatoid arthritis, congenital heart disease, blood clots in the pulmonary artery, HIV infection and liver disease. Diet drugs such as fenfluramine and dexfenfluramine (FenPhen) and the use of cocaine, methamphetamines or other street drugs are also causes.
Diagnosing pulmonary hypertension:
Tests include echocardiogram, electrocardiogram, pulmonary function tests (to measure air flow in and out of the lungs), perfusion lung scan (to show the blood flow in the lungs) and right-heart cardiac catheterization, which is the gold standard (to measure the blood pressure in the right side of the heart and the pulmonary artery).
Information:
Pulmonary Hypertension Association, 850 Sligo Ave., Suite 800, Silver Spring, MD 20910; (301) 565-3004; toll-free help line 1-800-748-7274.
Sources: Pulmonary Hypertension Association, Dr. John Swisher
Nancy Paradis can be reached at paradis@sptimes.com