Making Complex Adult Congenital Heart Disease a Little Simpler

Abstract

Advances in pediatric cardiothoracic surgery and cardiology have enabled most patients born with severe congenital cardiovascular malformations to survive into adulthood. Physicians are, therefore, increasingly likely to encounter adults with congenital heart disease (ACHD). Tetralogy of Fallot (TOF), 1 D-transposition of the great arteries (DTGA), 2 and single functional ventricle states 3 (eg, hypoplastic left heart syndrome) are three of the most prevalent of the complex cyanotic congenital defects, occurring in 17, 4, and 3 of 100,000 adults, respectively. 4 Most, but not all, patients with complex ACHD underwent palliative or reparative surgery early in life. As they survive into adulthood, they may require intervention to alter the expected course of disease progression, to treat complications of repair, or to address residual abnormalities. ACHD patients are also at increased risk of acquired heart disease. 5 Imaging plays a role in both diagnosis of symptomatic exacerbations and longterm maintenance. Imagers should become familiar with the underlying anatomy and pathophysiology of the common forms of ACHD, prior and current surgical repairs and their associated complications, as well as postoperative imaging guidelines. This article focuses on 3 ACHD complexes: TOF, DTGA, and single functional ventricle.