Adult Congenital Heart Disease

I t has long been recognized that manifestations of congenital heart disease extend beyond the cardiovascular system.The extracardiac ramifications of congenital heart disease are multifaceted and bidirectional.The intricate interplay between congenital heart physiology, bi-or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic effects are increasingly appreciated.Examples include the well-characterized neurological, hematologic, respiratory, skeletal, and hepatic consequences of cyanosis 1 ; thromboembolic complications in surgically palliated univentricular hearts 2 ; and dysregulation of the autonomic nervous system in various forms of congenital heart disease.3 Article p 2320In the present issue of Circulation, Dimopoulos and associates 4 provide original and important insights regarding the relationship between congenital heart disease and impaired renal function.They examine the prevalence of renal dysfunction across a wide-spectrum of adult congenital heart defects and explore implications on overall death rates.