Other Functionally Single Ventricle Lesions

This chapter outlines a wide morphologic spectrum of single ventricle lesions, a heterogenous group of disorders that exhibit parallel circulations at birth and converge to bidirectional cavopulmonary connection and Fontan palliation. Lesions can be categorized into those with obstruction to pulmonary flow, obstruction to systemic flow, unobstructed bilateral outflows, or balanced circulations. Initial physiology, clinical presentation, initial goals of medical stabilization, and surgical approach are defined by lesion category and the underlying physiology. There is considerable variability in long-term outcomes depending on native anatomy and associated cardiac lesions with estimated 10 to 15 year survival ranging from 50-75% for most patients with a ‘typical' risk profile. High risk subgroups include patients with a restrictive atrial septum, associated pulmonary venous anomalies, or those in which the majority of pulmonary blood flow is supplied by aortopulmonary collaterals.