Open AccessSurvival in Pulmonary Arterial Hypertension: A Brief Review of Registry Data
Author(s) -
Pauwaa Sunil,
Machado Roberto F.,
Desai Ank.it A.
Publication year - 2011
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.4103/2045-8932.87314
Subject(s) - medicine , patient registry , disease registry , pulmonary hypertension , disease , intensive care medicine , pediatrics
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 (Table 1). Survival in pulmonary arterial hypertension: A brief review of registry data