Adult congenital heart disease: toward prospective risk assessment of a multisystemic condition.

It 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, bior 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 cyanosis1; thromboembolic complications in surgically palliated univentricular hearts2; and dysregulation of the autonomic nervous system in various forms of congenital heart disease.3