Pulmonary arterial hypertension
Other Names for this Disease
- Familial pulmonary arterial hypertension
- Hereditary pulmonary arterial hypertension
- Heritable pulmonary arterial hypertension
- Idiopathic pulmonary arterial hypertension
How might pulmonary arterial hypertension be treated?
What is the long-term outlook for people with pulmonary arterial hypertension?
People with pulmonary arterial hypertension (PAH) benefit from receiving treatment at specialized centers. 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.
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).
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.
- Pulmonary arterial hypertension. Genetics Home Reference. January 2007; http://ghr.nlm.nih.gov/condition/pulmonary-arterial-hypertension. Accessed 9/28/2011.
- Rubin LJ, Hopkins W. Overview of pulmonary hypertension in adults. In: Basow DS. UpToDate. Waltham, MA: UpToDate; 2013;
- Hopkins W, Rubin LJ. Treatment of pulmonary hypertension in adults. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2013;