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Double-Outlet Ventricle Christoph Haller, Glen S Van Arsdell, Shi-Joon Yoo, Cecilia S.T GeorgeHyslop, Diane E Spicer, Robert H Anderson Abstract Double-outlet ventricle comprises a wide variety of malformations with an abnormal ventriculoarterial connection As variable as the morphology can be, so is the pathophysiologic picture and the therapeutic approach Successful treatment of patients with this congenital cardiac defect mandates a thorough understanding of the underlying anatomy and the physiologic changes characteristic of the various subtypes Patient presentation ranges from pulmonary overcirculation to well-balanced pulmonary and systemic blood flow to severe cyanosis Barely any other malformation covers such a wide spectrum of medical therapies, surgical procedures, and catheter interventions This chapter guides the reader from the morphology to the pathophysiologic aspects of double-outlet ventricle and highlights its clinical presentation, diagnosis, perioperative management as well as surgical and interventional strategies Keywords Double-outlet right ventricle; DORV; Double-outlet left ventricle; DOLV; Intraventricular communication; Subpulmonary; Subaortic; Doubly committed; Remote interventricular communication; Intraventricular streaming; Taussig-Bing anomaly Introduction Double-outlet ventricle is no more than an abnormal ventriculoarterial connection Therefore the feature may occur with each atrial arrangement, any atrioventricular connection, and all possible variations of ventricular morphology The morphologic arrangement can then be further complicated by associated defects, some common and others rare The clinical picture is as inconstant as the anatomic permutations and associations suggest This chapter reviews the various combinations in which the outflow tracts arise from the same ventricle but in the setting of concordant atrioventricular connections When both arterial trunks are found in the setting of discordant or mixed atrioventricular connections, the abnormal arrangements at either the atrioventricular or venoatrial junctions dominate the picture Double-outlet ventricle can also be found in the setting of the functionally univentricular heart
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