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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Pulmonary arterial hypertension


Other Names for this Disease
  • Familial pulmonary arterial hypertension
  • FPAH
  • Hereditary pulmonary arterial hypertension
  • Heritable pulmonary arterial hypertension
  • Idiopathic pulmonary arterial hypertension
More Names
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Your Question

My daughter has been diagnosed with familial pulmonary arterial hypertension & I was wondering if there is a cure & if not, how long will she have to live? Thank you.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Can pulmonary arterial hypertension be cured?

There are currently no targeted therapies that can reverse or stop pulmonary arterial hypertension (PAH). Transplantations of the lungs alone and lungs and heart have been performed in people with PAH. Unfortunately risk for life-threatening complications is high and long-term survival is limited.[1][2] Three year survival of those receiving transplantation is estimated to be around 50%.[2]

Last updated: 9/19/2013

How might pulmonary arterial hypertension be treated?

People with pulmonary arterial hypertension (PAH) benefit from receiving treatment at specialized centers.[2] The Pulmonary Hypertension Association offers a Find a Doctor tool which may aid you in locating your nearest center.

Treatment of serious or life threatening PAH may involve continuous IV epoprostenol. Other treatment options, include treprostinil, iloprost, bosentan, ambrisentan, sildenafil, and tadalafil. Many of these treatments can be administered in various forms, such as by shot, IV, or inhalation. A small number of people with PAH respond well to long term oral calcium channel blockers. Blood thinners, diuretics, and supplemental oxygen may be prescribed as needed.[2]

Many drugs can be harmful to people with PAH. The following should be avoided: appetite suppressants, cocaine, amphetamines (and related compounds), low oxygen environments (such as high altitudes), and possibly estrogen compounds (oral contraceptives and hormone replacement therapy).[2]

Last updated: 9/20/2013

What is the long-term outlook for people with pulmonary arterial hypertension?

The average survival of a person with pulmonary arterial hypertension (PAH) after diagnosis is estimated to be around 2.8 years, however individual prognosis may be better or worse depending on a variety of factors including age and severity of PAH. Studies have demonstrated survivals ranging from less than 1 year to over 7 years. Current treatments are helpful in controlling/managing symptoms experienced by people with PAH, but unfortunately no treatment has significantly improved survival.[2][3]

Last updated: 9/20/2013

References
  • Loyd JE, Phillips J. Heritable Pulmonary Arterial Hypertension. GeneReview. Revised December 20, 2013; http://www.ncbi.nlm.nih.gov/books/NBK1485/. Accessed 9/19/2013.
  • Hopkins W, Rubin LJ. Treatment of pulmonary hypertension in adults. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2013;
  • Rubin LJ, Hopkins W. Overview of pulmonary hypertension in adults. In: Basow DS. UpToDate. Waltham, MA: UpToDate; 2013;