Congenital Heart Disease-Associated Pulmonary Hypertension

Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in children and adults. This increased prevalence is seen despite significant advances in early diagnosis and surgical correction of patients with structural CHD. PAH is the cause of significant morbidity and mortality in these patients and comes in many forms. With the increased availability of targeted therapies for PAH, there is hope for improved hemodynamics, exercise capacity, quality of life, and possibly survival for these patients. There may also be opportunities for combined medical and interventional/surgical approaches for some.