ABSTRACT
Increasingly effective surgical therapies for congenital heart disease (CHD) over the past four decades have caused a rapid increase in the number of adults who have some form of CHD (1). Many of the adult patients managed in congenital heart centers continue to have pulmonary hypertension caused by unrepaired systemic to pulmonary shunts. Pulmonary hypertension also continues to be a significant problem in pediatric patients with repaired, palliated, and unrepaired CHD. While CHD patients are predisposed to have high pulmonary vascular resistance because of excessive pulmonary blood flow and/or high left atrial pressures, patients with “single ventricle” CHD physiology require especially low pulmonary vascular resistance so that they can benefit from cavopulmonary shunts (the Glenn and Fontan operations).
