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Double-Inlet Single Ventricle
Introduction
Double-inlet single ventricle is a rare anomaly, accounting for 2% to 3% of all congenital heart diseases (CHDs). Double-inlet single ventricle comprises a heterogeneous group of cardiac anomalies characterized by the presence of two atria with a single ventricle. Cardiac and extracardiac anomalies are commonly associated. The prognosis is usually very poor, and univentricular repair is required.
Disease
Definition
Double-inlet single ventricle comprises a heterogeneous group of cardiac anomalies characterized by two atria and a single ventricle with one or two atrioventricular valves. Although a rudimentary accessory ventricle may also exist, only one ventricle is functional, receiving the flow from both atria. This entity does not include cases with a single functional ventricle secondary to mitral or tricuspid atresia.
Prevalence and Epidemiology
Double-inlet single ventricle is a rare anomaly. It occurs in approximately 0.5 : 10,000 to 1.0 : 10,000 live births and accounts for 2% to 3% of all CHDs.
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