pulmonary arterial hypertension, PAH

pulmonary arterial hypertension, PAH

A rare disorder featuring a rise in arterial pressure in the pulmonary vessels in the absence of secondary causes. The precapillary pulmonary arteries are affected by intimal fibrosis and medial hypertrophy. Patients present with breathlessness on minor effort or indications of right heart failure. The prognosis is poor, with half of the untreated patients dying within three years of diagnosis. Treatments to prolong life include epoprostenol, endoththelin-1-receptor antagonists and phosphodiesterase inhibitors.

Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005