Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy

Pulmonary arterial hypertension (PAH) associated with congenital heart disease remains a major problem despite advances in cardiac surgery. Recently, advanced therapies for PAH have become available and have been effective in reducing pulmonary vascular resistance and symptoms in patients with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease. This has led to a new dilemma, namely could intracardiac communications previously considered inoperable due to severe pulmonary vascular disease become amenable to surgery after successful treatment with advanced therapy? We address, hereby, the potential merits and hazards of a “treat-and-repair” approach using advanced therapies in patients with PAH associated with congenital heart disease.