Primary pulmonary hypertension (PPH) is a syndrome of dyspnea, chest pain, and syncope
defined by increased pulmonary vascular resistance and the absence of a known cause. It …

Pulmonary arterial hypertension (PAH) is a progressive and fatal disease for which there is
an ever-expanding body of genetic and related pathophysiological information on disease …

Primary pulmonary hypertension (PPH), characterized by obstruction of pre-capillary
pulmonary arteries, leads to sustained elevation of pulmonary arterial pressure (mean> 25 …

Familial primary pulmonary hypertension is a rare autosomal dominant disorder that has
reduced penetrance and that has been mapped to a 3-cM region on chromosome 2q33 …

Background Most patients with familial primary pulmonary hypertension have defects in the
gene for bone morphogenetic protein receptor II (BMPR2), a member of the transforming …

BACKGROUND Primary pulmonary hypertension (PPH), resulting from occlusion of small
pulmonary arteries, is a devastating condition. Mutations of the bone morphogenetic protein …

Primary pulmonary hypertension (PPH) is a potentially lethal disorder, because the
elevation of the pulmonary arterial pressure may result in right-heart failure. Histologically …

Primary pulmonary hypertension (PPH) is a frequently fatal disease whose pathobiology is
poorly understood. Monoclonal endothelial cell growth is present within plexiform lesions of …

Transforming growth factor-β (TGF-β) regulates many cellular processes through complex
signal-transduction pathways that have crucial roles in normal development. Disruption of …

Mutations in two receptors of the transforming growth factor-beta family have recently been
shown to be present in the majority of cases of inherited (familial) pulmonary arterial …