font size

Pulmonary Hypertension (cont.)

Medical Author:
Medical Author:
Medical Editor:

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

Patient Comments

Viewers share their comments

Pulmonary Hypertension - Symptoms Question: Please describe your symptoms of pulmonary hypertension.
Pulmonary Hypertension - Treatments Question: What was the treatment for your pulmonary hypertension?
Pulmonary Hypertension - Share Your Experience Question: Were you or a relative diagnosed with pulmonary hypertension? Please share your experience.
Source: MedicineNet.com
http://www.medicinenet.com/pulmonary_hypertension/article.htm

Women's Health

Find out what women really need.

Pulmonary Hypertension Related Articles
advertisement
advertisement
Use Pill Finder Find it Now See Interactions

Pill Identifier on RxList

  • quick, easy,
    pill identification

Find a Local Pharmacy

  • including 24 hour, pharmacies

Interaction Checker

  • Check potential drug interactions
Search the Medical Dictionary for Health Definitions & Medical Abbreviations