Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance is a frequent complication of congenital heart disease, most commonly occurring with systemic-to-pulmonary shunt lesions. The natural disease progression involves pulmonary endothelial damage due to exposure to increased pulmonary blood flow and pressure, and in its most severe form results in Eisenmenger syndrome, in which there is shunt reversal and cyanosis. Because of anatomic and pathologic similarities of PAH in the congenital heart disease population compared with idiopathic PAH, there is an evolving role for the use of the newer targeted PAH therapies in these patients. While early closure of shunt lesions is the best preventive measure, the concept of a combined medical-surgical approach for these patients has emerged as well. An additional group of patients that may benefit from the use of targeted PAH therapies includes the rapidly growing subpopulation of patients who have undergone the Fontan operation due to single ventricle anatomy. © 2013, Springer Science + Business Media New York.