Molecular mechanisms of pulmonary arterial hypertension: role of mutations in the bone morphogenetic protein type II receptor
RJ Davies, NW Morrell - Chest, 2008 - Elsevier
Pulmonary arterial hypertension (PAH) is characterized by abnormal remodeling of small,
peripheral resistance vessels in the lung involving proliferation and migration of vascular
smooth muscle, endothelial cell and fibroblasts. The increase in pulmonary vascular
resistance leads to right heart failure, and, without treatment, death occurs within 3 years of
diagnosis. The etiology of PAH is multifactorial. In some patients, there is a major genetic
predisposition in the form of heterozygous germline mutations in a transforming growth …
peripheral resistance vessels in the lung involving proliferation and migration of vascular
smooth muscle, endothelial cell and fibroblasts. The increase in pulmonary vascular
resistance leads to right heart failure, and, without treatment, death occurs within 3 years of
diagnosis. The etiology of PAH is multifactorial. In some patients, there is a major genetic
predisposition in the form of heterozygous germline mutations in a transforming growth …