Does Bone Morphogenetic Protein Receptor Type 2 (BMPR2) mutation affect RV function and signaling pathways in patients with pulmonary arterial hypertension?
BMPR2 mutation carriers with PAH have worse RV function than noncarriers, which is not explained by differences in TGF-β or BMPR2 signaling.
Despite a similar afterload, RV function is more severely affected in mutation carriers than in noncarriers. However, these differences cannot be explained by a differential transforming growth factor β, bone morphogenetic protein receptor II signaling, or cardiac adaptation.
Bruggen et al. (Mon,) studied this question.