ABSTRACT
Some patients with a functionally single ventricle may survive without surgery until adulthood, in the presence of favorable hemodynamics and balanced pulmonary artery flow associated with significant pulmonary stenosis. Under certain circumstances, Fontan procedure or some of its modifications can be performed, even in adulthood. This chapter describes the most common complex congenital heart diseases (CHD) with univentricular circulation, which may be surgically managed by Fontan circulation, usually total cavo-pulmonary connection (TCPC). Tricuspid atresia (TA) accounts for some 0.7% of all CHD. In TA, the tricuspid valve is absent, and there is no anatomic connection and direct communication between the right atrium and right ventricle; a fibrotic septum is usually present at the site of the tricuspid valve. Double-outlet right ventricle encompasses virtually the entire spectrum of congenital cardiac anatomy and physiology. Both great arteries arise predominantly (>50%) from the morphologically right ventricle.
