Primary Pulmonary Hypertension
What is primary pulmonary hypertension (PPH)?
Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels. It is usually inherited or caused by unknown reasons.
History of the disease:
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 new cases diagnosed each year. It is most common in women between the ages of 20 and 40; however, anyone can develop it.