Functionally Univentricular Heart with Right Heart Hypoplasia (Tricuspid Atresia with Normally Related Great Arteries and Pulmonary Atresia with Intact Ventricular Septum)

Abstract

Univentricular hearts can present with a hypoplastic left-sided ventricle, a hypoplastic right-sided ventricle, or a ventricle with ambiguous morphology. This chapter will focus on the various forms of hypoplastic right ventricle. The most common forms of this defect are tricuspid atresia and pulmonary valvar atresia with an intact ventricular septum. Almost all forms of hypoplastic right heart syndrome are treated with a series of three operations. The first operation typically either augments or restricts pulmonary blood flow, depending on the specific anatomy. The second stage consists of redirecting superior vena cava flow directly to the lungs. The final operation (Fontan procedure) consists of redirecting the remainder of systemic venous return directly to the lungs. The end result is an in series circulation, with separate pulmonary and systemic circulations.