Double Inlet Ventricle

Significant controversy exists regarding the definition and nomenclature used to describe hearts that have a large dominant ventricle and a smaller incomplete ventricle or chamber. Functionally univentricular lesions involving atresia of one of the atrioventricular valves will be described in other chapters. In this chapter, we focus specifically on hearts with double inlet atrioventricular connection, in other words hearts unified because both atrial chambers are committed to the same dominant ventricle. The morphology of the various subtypes will be described, as well as the pathophysiology and clinical presentation of each subtype. Diagnostic evaluation and subsequent management will also be discussed.