The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease.

A significant number of adult patients with congenital heart disease suffer from pulmonary arterial hypertension leading to a markedly increased morbidity and mortality. Some defects may be eligible for operative or interventional repair in adulthood but careful selection of candidates is crucial. With the emergence of disease-targeting therapies, symptomatic improvement and stabilization have become possible while the impact on survival currently remains unclear.