Heritable Pulmonary Arterial Hypertension

**Semantic type:** Disease or Syndrome

**Definition:** A rare, progressive, autosomal dominant inherited disorder. It is caused by mutation in the BMPR2 gene in most cases. It is characterized by abnormally high blood pressure in the pulmonary artery, caused by obstruction and obliteration of the small pulmonary arteries.

**Synonyms:** - HPAH