Single-stage repair of Scimitar variant with Swiss cheese ventricular septal defects without deep hypothermic circulatory arrest

ABSTRACT

Scimitar variant is a rare partial anomalous pulmonary venous connection that lacks right lung hypoplasia and cardiac dextroposition. Its coexistence with Swiss cheese-type ventricular septal defects (VSDs) is extremely rare and can result in complex hemodynamic changes. A 23-month-old boy with Scimitar variant and multiple Swiss cheese-type VSDs underwent single-stage repair. Preoperative imaging revealed anomalous drainage of the entire right pulmonary venous return into the inferior vena cava and a persistent left superior vena cava. The dominant perimembranous defect was patched, while smaller muscular defects were managed conservatively. The repair was performed without deep hypothermic circulatory arrest.