Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease

Abstract

Congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation. CHD-associated PAH has an estimated prevalence of 5–10% in adult patients, with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension (PAH)-targeted pharmacotherapy. Although limited data exist, targeted PAH pharmacotherapy has proven to be beneficial in patients with CHD-associated PAH, with observed improvement in functional class, increase in exercise capacity, and improvement in quality of life and cardiopulmonary hemodynamics. Additionally, there has been increasing interest in the “treat-to-close” strategy. PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’ operability in repairing the cardiac defect. Although there have been significant advances in the management of this disease state in the past 2 decades, mortality remains high, and ongoing clinical trials are needed to better understand the treat-to-close strategy.