Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is high blood pressure in the arteries of the lungs. The condition narrows, blocks, or destroys arteries in the lungs. This makes the heart work harder than normal to push blood through the lungs. The extra work puts strain on the heart. Eventually, the heart muscle becomes weak and fails.(1) About 75 percent of heritable pulmonary arterial hypertension (HPAH) have an identified mutation. HPAH has been linked to mutations in the BMPR2 gene in 75 percent of cases. Other genes linked to HPAH include ACVR1, BMPR1, CAV1, ENG and SMAD9, and CBLN2. However, most people carrying an HPAH mutation do not develop PAH. Idiopathic pulmonary arterial hypertension (IFPAH) is the name for pulmonary arterial hypertension with an unknown cause.