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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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Treatment


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How might pulmonary arterial hypertension be treated?

People with pulmonary arterial hypertension (PAH) benefit from receiving treatment at specialized centers.[1] 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.[1]

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).[1]

Last updated: 9/20/2013

References
  1. Hopkins W, Rubin LJ. Treatment of pulmonary hypertension in adults. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2013;


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Pulmonary arterial hypertension. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name Riociguat
Trade Name
(Manufacturer Name)
Adempas
(Bayer HealthCare Pharmaceuticals, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of adults with pulmonary arterial hypertension (PAH) WHO Group 1, to improve exercise capacity, WHO functional class and to delay clinical worsening.
More Information about this product Drug Information Portal

Generic Name Epoprostenol
Trade Name
(Manufacturer Name)
Flolan®
(Glaxo Wellcome Inc.)
Indication
The FDA has approved this product to be used in this manner.
Long-term intravenous treatment of primary pulmonary hypertension in NYHA Class III and Class IV patients.
More Information about this product Drug Information Portal

Generic Name Ambrisentan
Trade Name
(Manufacturer Name)
Letairis®
(Gilead Colorado)
Indication
The FDA has approved this product to be used in this manner.
Treatment of pulmonary arterial hypertension (WHO group I) in patients with WHO class II or III symptoms to improve exercise capacity and delay clinical worsening
More Information about this product Drug Information Portal

Generic Name macitentan
Trade Name
(Manufacturer Name)
Opsumit
(Actelion Pharmaceuticals Ltd)
Indication
The FDA has approved this product to be used in this manner.
Treatment of pulmonary arterial hypertension (PAH, WHO Group 1) to delay disease progression. Disease progression included: death, initiation of intravenous (IV) or subcutaneous prostanoids, or clinical worsening of PAH (decreased 6-minute walk distance, worsened PAH symptoms and need for additional PAH treatment). Opsumit also reduced hospitalization for PAH.
More Information about this product Drug Information Portal

Generic Name Treprostinil
Trade Name
(Manufacturer Name)
Remodulin®
(United Therapeutics Corp.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of pulmonary arterial hypertension.
More Information about this product Drug Information Portal

Generic Name Bosentan
Trade Name
(Manufacturer Name)
Tracleer®
(Actelion Life Sciences Ltd.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of pulmonary arterial hypertension.
More Information about this product Drug Information Portal
Medline Plus Health Information

Generic Name Iloprost solution for infusion
Trade Name
(Manufacturer Name)
Ventavis®
(Acetlion)
Indication
The FDA has approved this product to be used in this manner.
Treatment of pulmonary arterial hypertension (WHO Group I) in patients with NYHA Class III or IV symptoms
More Information about this product Drug Information Portal