Pulmonary Hypertension (cont.)

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What causes primary pulmonary hypertension?

Primary pulmonary hypertension has no identifiable underlying cause. Primary pulmonary hypertension is also referred to as idiopathic pulmonary hypertension. Primary pulmonary hypertension is more common in young people and more common in females than males.

Primary pulmonary hypertension is an unusually aggressive and often fatal form of pulmonary hypertension. Whereas it is known that the arterial obstruction is caused by a building up of the smooth muscle cells that line the arteries, the underlying cause of the disease has long been a mystery.

A genetic cause of the familial form of primary pulmonary hypertension has been discovered. It is caused by mutations in a gene called BMPR2. BMPR2 encodes a receptor (a transforming growth factor beta type II receptor) that sits on the surface of cells and binds molecules of the TGF-beta superfamily. Binding triggers conformational changes that are shunted down into the cell's interior where a series of biochemical reactions occur, ultimately affecting the cell's behavior. The mutations block this process. This discovery may provide a means of genetic diagnosis and a potential target for the therapy of people with familial (and possibly also sporadic) primary pulmonary hypertension.

How common is pulmonary hypertension?

There are no clear statistical data on the prevalence of pulmonary hypertension. Since pulmonary hypertension is seen in multiple different conditions, it is perhaps better to look at the prevalence in each condition. It is fairly common in patients with heart failure, COPD, and sleep apnea. It occurs in 7% to 12% of patients with collagen vascular disease and in less than 5% of HIV patients.

Medically Reviewed by a Doctor on 11/25/2013

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