Unoperated Congenitally Corrected Transposition of the Great Arteries, Nonrestrictive Ventricular Septal Defect, and Pulmonary Stenosis in Middle Adulthood: Do Multiple Wrongs Make a Right?

Submitted May 6, 2011; Accepted August 3, 2011. The survival into adulthood of patients with unoperated complex congenital heart disease with anomalies often considered life threatening in infancy and childhood requires a complex interplay of “balanced” defects allowing for cardiovascular physiology compatible with long-term survival. We report on a series of three cases from our advanced imaging database of middle-aged adults presenting with multiple similar defects providing a hemodynamically balanced circulation. The constellation of defects seen in each of these patients included congenitally corrected transposition of the great arteries, a large nonrestrictive ventricular septal defect, valvular pulmonary stenosis, and in two cases anomalous coronary arteries. Cardiovascular computed tomographic angiography (CCTA) and cardiovascular magnetic resonance imaging (CMR) were important to the characterization of the multiple defects and their three-dimensional relationships in these cases. Treatment decisions in patients with this constellation of findings are challenging, given the limited data due to the rarity of survival of patients with these defects into middle adulthood and the paucity of data related to decisions and approaches to medical management, surgical correction, or transplantation.