A Blue Baby Grows Up

This report describes the history of a 41-year-old female patient born with atresia of the right atrioventricular valve, hypoplasia of the right ventricle, an atrial septal defect, and malposed great arteries. The patient underwent surgical pulmonary artery banding in infancy but did not undergo corrective surgery due to the development of pulmonary arterial hypertension. This report touches on several important clinical issues that may arise when caring for the adult with complex palliated cyanotic congenital heart disease, including the development and treatment of pulmonary arterial hypertension.