Survival in Pulmonary Arterial Hypertension: A Brief Review of Registry Data

PVRI Member Authors: Ankit Desai


Pulmonary arterial hypertension (PAH) is a severe and fatal disease with a prevalence of 15 cases in a million[1] and characterized by increased pulmonary vascular resistance and right heart failure. PAH can be idiopathic (IPAH), familial (FPAH), or associated with other disorders. In 1984 the National Institute of Health (NIH) compiled the first large registry of PAH patients (all IPAH, FPAH, and anorexigen-associated) confirming poor survival and prognosis.[2] In an era of PAH-specific therapies, subsequent PAH registries (the Pulmonary Hypertension Connection [PHC] registry,[3] the French registry,[4] and the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management [REVEAL][5]) have demonstrated improved prognosis.

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Pulmonary Circulation Vol 1: No 3 cover image

September 2011

Pulmonary Circulation Vol 1: No 3

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