Endothelin receptor antagonists for pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a devastating disease, which leads to right heart failure and premature death. Pulmonary arterial hypertension can be classified into five categories according to Venice classification: (1) Idiopathic PAH; (2) Familial PAH; (3) PAH associated with collagen vascular disease, congenital systemic-to-pulmonary shunts, portal hypertension, HIV infection, drugs and toxins or other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy); (4) PAH associated with significant venous or capillary involvement, which includes pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH); (5) Persistent pulmonary hypertension of the newborn. PAH can also be secondary to chronic hypoxic lung disease as part of the "cor-pulmonale" syndrome, and also secondary to left sided heart disease, but these conditions are usually distinguished from those listed here.