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Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels.
The first reported case of primary pulmonary hypertension occurred in 1891 with a published description of an autopsy that showed thickening of the deceased's pulmonary artery, but no indications of heart or lung disease. In 1951, when 39 cases were reported in the United States, the illness received its name.
Between 1967 and 1973, an unexplained increase in primary pulmonary hypertension was reported in central Europe. The increase in the number of cases was eventually attributed to aminorex fumarate, an amphetamine-like drug introduced in Europe in 1965 to control appetite. When aminorex was removed from the market, the incidence of primary pulmonary hypertension went down to normal levels.
More recently, in the United States and France, primary pulmonary hypertension has been associated with the appetite suppressants fenfluramine and dexfenfluramine, which were taken off the market in 1997. In the US, there are an estimated 300 to 1,000 new cases diagnosed each year, but the actual number of cases is unknown. It is most common in women between the ages of 20 and 40; however, anyone can develop it.
The exact cause of PPH is unknown. Research has linked primary pulmonary hypertension to genetic or familial predisposition. Researchers believe the blood vessels are particularly sensitive to certain internal or external factors, and constrict, or narrow, when exposed to these factors, such as an immune system factor, or sensitivity to drugs or other chemicals.
Secondary pulmonary hypertension occurs as a result of the effects of other conditions which may include diseases of the heart or lungs, a blood clot in the lungs, or a condition called scleroderma.
The following are the most common symptoms for pulmonary hypertension. However, each individual may experience symptoms differently. Symptoms may include:
More severe symptoms indicate a more advanced disease. In advanced stages, the patient:
The symptoms of primary pulmonary hypertension may resemble other conditions or medical problems. Consult your physician for a diagnosis.
Pulmonary hypertension is rarely discovered in a routine medical examination, and in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.
Pulmonary hypertension is a diagnosis of exclusion. Diagnostic procedures may include:
Specific treatment will be determined by your physician based on:
Treatment may include one or more of the following: