Univentricular heart: management options

The term ''Univentricular Heart'' encompasses a wide variety of heart defects that functionally and physiologically constitute a single ventricular chamber. The terminology ''univentricular repair'' is frequently used in the surgical literature to include those biventricular hearts that are not amenable for a final two ventricle repair and need to go through the same surgical stages as with a functionally univentricular heart, culminating finally in a total cavo-pulmonary connection. Broadly, treatment is focused on controlling the pulmonary blood flow in early infancy, by means of aorto-pulmonary shunting in pulmonary atresia or stenosis, and pulmonary artery banding or pulmonary artery disconnection with aorto-pulmonary shunt placement in high pulmonary blood flow situations. Concomitant repair of other associated conditions is required. Babies with hypoplastic left heart physiology undergo staged "Norwood" repair resulting in an eventual total cavo-pulmonary connection with the RV functioning as the systemic ventricle. In this review, medical and surgical management of these patients will be discussed, after a brief discussion of nomenclature, anatomic and physiologic considerations.