July 24, 2016
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Pulmonary Hypertension (cont.)

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What causes idiopathic pulmonary (primary) pulmonary hypertension?

Primary pulmonary hypertension has no identifiable underlying cause. This term is mainly a historical name and no longer used. The current term used to describe this condition is idiopathic pulmonary hypertension. Idiopathic pulmonary hypertension is more common in young people and more common in females than males.

Idiopathic pulmonary hypertension unusually is an 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 idiopathic pulmonary hypertension, now referred to as Heritable PAH, 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.

What kind of doctor treats pulmonary hypertension?

Generally, pulmonary physicians (pulmonologists) and cardiologists treat pulmonary hypertension. Depending on the cause, other physicians also may be involved such as sleep medicine doctors, rheumatologists, and perhaps even infectious disease specialists. Today, more health-care professionals in the community are becoming adept at managing pulmonary hypertension. However, it is reasonable to get a second opinion regarding management at a tertiary, university setting because the field of management of pulmonary artery hypertension is evolving rapidly.

Medically Reviewed by a Doctor on 3/30/2016

Source: MedicineNet.com
http://www.medicinenet.com/pulmonary_hypertension/article.htm

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