Endothelin receptor antagonist therapy in congenital heart disease with shunt-associated pulmonary arterial hypertension: A qualitative systematic review

摘要

BACKGROUND:Congenital heart disease (CHD) with systemic-to-pulmonary shunting is associated with pulmonary arterial hypertension (PAH). There are similar clinical and pathophysiological features between CHD with shunt-associated PAH and idiopathic PAH. Endothelin-receptor antagonists (ERAs) are oral medications that improve pulmonary hemodynamics, symptoms and functional capacity in many PAH patients. However, the role of ERAs in CHD with shunt-associated PAH is unclear.