Summary
Pulmonary arterial hypertension (PAH) is a term that refers to a group of rare diseases where the blood pressure in the arteries of the lungs (pulmonary arteries) becomes abnormally high.
The term “pulmonary hypertension” is more general and refers to high blood pressure in the pulmonary arteries. Pulmonary arterial hypertension (PAH), the subject of this report, is a specific type of pulmonary hypertension where the pulmonary arteries become narrowed, thickened, or blocked. Not all forms of pulmonary hypertension are PAH.
The pulmonary arteries carry blood from the right side of the heart to the lungs, where it picks up oxygen. In PAH, the arteries become narrow or blocked, increasing pressure. This puts a strain on the right side of the heart, which must work harder to pump blood. If not treated, over time, this can lead to right heart failure.
PAH usually affects females between the ages of 30-60. People with PAH may go years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise.
The cause is unknown in many cases (idiopathic). It can also be inherited or familial (heritable) or it may be caused by drugs or toxins or be associated with other medical conditions.
About 15-20% of people with PAH have heritable forms of PAH. Research has found gene changes (variants) in people with heritable PAH or with other forms of PAH, in particular a variant in the BMPR2 gene. However, in some people, even when there are several people with PAH in a family, no gene variant is identified.
PAH is a progressive and can be a very severe and potentially fatal condition if it is not treated. The progressive nature of this disease means that an individual may have only mild symptoms at first but will eventually require treatment and medical care to maintain a reasonable quality of life. Although treatable, there is no known cure for the disease. Treatment may include medications to open lung arteries (vasodilators), oxygen therapy, water pills (diuretics) for fluid buildup, blood thinners to prevent clots and lung or heart-lung transplants for advanced cases. Early diagnosis is very important for better outcomes.
Introduction
The first case of PAH was described in 1891 by German physician E. Romberg, who noted thickening of the pulmonary artery without any clear heart or lung disease. In 1951, Dr. D.T. Dresdale reported three more cases and used the term primary pulmonary hypertension, which is no longer used but refers to what we now classify under idiopathic PAH.
In the 1990s, studies linked PAH to diet pills like Fen-Phen, Pondimin, and Redux, which were removed from the market in 1997. Other drugs such as methamphetamines, have also been associated with PAH.
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