Pulmonary arterial hypertension (PAH) is an uncommon but serious disease characterized by severe pulmonary
vascular disease and significant morbidity and mortality. PAH associated with
congenital heart disease (
APAH-CHD) is one etiology of PAH that has innate characteristics delineating it from other forms of PAH. The patient with
APAH-CHD presents with unique challenges consisting of not only pulmonary
vascular disease but also the complexity of the cardiac lesion.
Eisenmenger syndrome (ES) represents the severe end of the spectrum for disease in
APAH-CHD. Over time, systemic-to-pulmonary shunting through cardiac defects increases pulmonary vascular resistance to levels significant enough to reverse shunting across the defect. Historically, ES patients have been reported to have better outcomes than IPAH despite similarities in pulmonary
vascular disease. However, recent studies are challenging this notion. Nonetheless,
APAH-CHD survival has improved with the advent of modern PAH targeted
therapies. New therapeutic options have allowed us to reconsider the dogma of inoperability in
APAH-CHD patients with unrepaired defects. Certainly advances have been made, however, investigators must continue to advance the field through controlled clinical trials in both adult and pediatric
APAH-CHD patients.