Article
Pulmonary Hypertension Induced by Right Pulmonary Artery Occlusion: Hemodynamic Consequences of Bmpr2 Mutation.
Journal of the American Heart Association - 16 Jul 2024
Todesco Alban, Grynblat Julien, Akoumia Kouamé Kan Firmin, Bonnet Damien, Mendes-Ferreira Pedro, Morisset Stéphane, Chemla Denis, Levy Marilyne, Méot Mathilde, Malekzadeh-Milani Sophie-Guiti, Tielemans Birger, Decante Benoit, Vastel-Amzallag Carine, Habert Paul, Ghigna Maria-Rosa, Humbert Marc, Montani David, Boulate David, Perros Frédéric
Abstract excerpt
BACKGROUND: The primary genetic risk factor for heritable pulmonary arterial hypertension is the presence of monoallelic mutations in the BMPR2 gene. The incomplete penetrance of BMPR2 mutations implies that additional triggers are necessary for pulmonary arterial hypertension occurrence. Pulmonary artery stenosis directly raises pulmonary artery pressure, and the redirection of blood flow to unobstructed...
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