50?mL) was significantly more frequent in BMPR2 mutation carriers. 43.5% of BMPR2 mutation carriers, as opposed to 9.5% of noncarriers, displayed singular large fibrovascular lesions, which appear to be closely related to the systemic lung vasculature. Our analysis provides evidence for the involvement of the pulmonary systemic circulation in BMPR2 mutation-related PAH. We show that BMPR2 mutation carriers are more prone to haemoptysis and that haemoptysis is closely correlated to bronchial arterial remodelling and angiogenesis; in turn, pronounced changes in the systemic vasculature correlate with increased pulmonary venous remodelling, creating a distinctive profile in PAH patients harbouring a BMPR2 mutation."">

BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension

Maria-Rosa Ghigna, Christophe Guignabert, David Montani, Barbara Girerd, Xavier Jaïs, Laurent Savale, Philippe Hervé, Vincent Thomas de Montpréville, Olaf Mercier, Olivier Sitbon, Florent Soubrier, Elie Fadel, Gérald Simonneau, Marc Humbert, Peter Dorfmüller

Source: Eur Respir J 2016; 48: 1668-1681
Journal Issue: December
Disease area: Pulmonary vascular diseases

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Maria-Rosa Ghigna, Christophe Guignabert, David Montani, Barbara Girerd, Xavier Jaïs, Laurent Savale, Philippe Hervé, Vincent Thomas de Montpréville, Olaf Mercier, Olivier Sitbon, Florent Soubrier, Elie Fadel, Gérald Simonneau, Marc Humbert, Peter Dorfmüller. BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension. Eur Respir J 2016; 48: 1668-1681

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