Article
BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis.
The Lancet. Respiratory medicine - 1 Feb 2016
Evans Jonathan D W, Girerd Barbara, Montani David, Wang Xiao-Jian, Galiè Nazzareno, Austin Eric D, Elliott Greg, Asano Koichiro, Grünig Ekkehard, Yan Yi, Jing Zhi-Cheng, Manes Alessandra, Palazzini Massimiliano, Wheeler Lisa A, Nakayama Ikue, Satoh Toru, Eichstaedt Christina, Hinderhofer Katrin, Wolf Matthias, Rosenzweig Erika B, Chung Wendy K, Soubrier Florent, Simonneau Gérald, Sitbon Olivier, Gräf Stefan, Kaptoge Stephen, Di Angelantonio Emanuele, Humbert Marc, Morrell Nicholas W
Abstract excerpt
BACKGROUND: Mutations in the gene encoding the bone morphogenetic protein receptor type II (BMPR2) are the commonest genetic cause of pulmonary arterial hypertension (PAH). However, the effect of BMPR2 mutations on clinical phenotype and outcomes remains uncertain. METHODS: We analysed individual participant data of 1550 patients with idiopathic, heritable, and anorexigen-associated PAH from eight cohorts that...
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