Double-Outlet Left Ventricle

newborn male was transferred for severe cyanosis and suspected transposition of the great arteries and ventric- ular septal defect (VSD). An emergency balloon septostomy was performed. The position and commitment of VSD and great arteries were more precisely defined by echocardiogra- phy. There was double-outlet left ventricle (DOLV) with doubly committed VSD, l-malposition of the great arteries, and pulmonary stenosis. The infundibular septum was virtu- ally absent. A modified Blalock-Taussig shunt was inserted before a Rastelli-type corrective surgery was performed at 20 months of age. The VSD was closed, the pulmonary trunk was transsected, and a right ventricle-pulmonary artery conduit was placed. Intraoperative findings confirmed the diagnosis. Perioperative assessment was done noninvasively (Figures 1 and 2 and Movies I and II). Mild subaortic stenosis was found and attributed to bulging of the VSD patch. Otherwise, the intracardiac findings were excellent. When the patient was 5 years old, the conduit became stenotic. Again, the intra- and extracardiac anatomy, including the conduit's aspect and its relation to the sternum, could be seen clearly with the use of noninvasive imaging techniques (Figure 3 and Movie III). The postoperative course has been uneventful. The boy is now 6 years old. In contrast to double-outlet right ventricle, DOLV is a rarity. After some dispute, its existence was finally verified intraoperatively and in autopsy series.1 DOLV with doubly committed VSD and l-malposed great arteries comprises fewer than 10% of cases in the largest series.2,3 None of theses patients had pulmonary stenosis. The anatomic fea- tures of this case may not be compatible with traditional