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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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Prognosis


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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.[1][2]

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