Targeted Pulmonary Arterial Hypertension Therapies and a Combined Medical-Surgical Approach for Congenital Heart Disease Patients

Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance (PVR) is a frequent complication of congenital heart disease (CHD), 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 (ES), in which there is shunt reversal and cyanosis. Due to anatomic and histopathologic similarities of PAH in patients with CHD, as well as those with idiopathic and other forms of Group 1 PAH, there is an evolving interest and role for the use of the newer targeted PAH therapies in CHD patients. While early closure of shunt lesions is the best preventive measure, the use of targeted medical therapies when a patient is too high risk for surgery, following surgical repair, and even in those with reversal of shunting due to advanced disease, has emerged over the past decade.