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Patients with complex systemic and pulmonary venous anatomy, common atrioventricular canal defects and conotruncal anomalies have traditionally been routed to univentricular palliation and labeled as "unseptatable." This report describes our initial experience in septation/biventricular conversion ("ventricular switch"), utilizing the left ventricle (LV) as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries. Five consecutive patients with challenging anatomic configuration underwent septation. All patients were severely cyanotic and had important functional limitations. All patients required complex atrial septation. Ventricular septation was precluded by fixed pulmonary vascular resistance in 2 patients. Systemic venous return was diverted to the morphologic LV as part of physiological 2V (n = 4) or 1.5 V repair (n = 1). Median conversion age was 9 years (range 11 monthsÀ46 years). Four patients had 12 previous cardiac surgical procedures in preparation for univentricular repair elsewhere. Three dimensional-printed heart models evaluated feasibility of septation. All patients are alive at a median follow-up of 0.6 years (range 0.08À2.7 years). Median hospital stay was 13 (range 10À60) days. LV recruitment improved functional status and significantly increased systemic oxygen saturation in all patients (79 § 7% vs 95 § 5%, P = 0.003). We report a novel paradigm for successfully utilizing both ventricles with the morphologic LV as the subpulmonary ventricle, in a complex population thought to be unseptatable. This approach is versatile and can likely be extrapolated to other complex anatomic configurations. Although we utilized this strategy in patients of variable age, earlier ventricular switch may yield the best results.

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava (IVC) creating a tubular opacity paralleling the right cardiac border on chest radiography which resembles a curved Turkish sword or scimitar. Associated pulmonary and vascular anomalies have been reported in cases of Scimitar syndrome, most commonly hypoplasia of right lung, dextroposition of the heart, hypoplasia of the right pulmonary artery, and aberrant arterial supply from the descending aorta to the affected lobe of the right lung. To the best of our knowledge, this is the first case of Scimitar syndrome with an H-type tracheoesophageal fistula that has ever been reported.

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