Pediatric pulmonary arterial hypertension--a review.

Pulmonary arterial hypertension (PAH) afflicts thousands of children worldwide. The pathophysiology involves intravascular proliferation and remodeling leading to an increase in pulmonary vascular resistance which if left untreated results in right heart failure and death. Signs and symptoms are subtle as the disease progresses to irreversible lung damage. There is no cure for PAH, however newer methods of treatment can successfully manage these patients and delay progression of the disease process.