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Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative twodimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

Surgical approaches for transposition of the great arteries with aortic arch obstruction include primary repair and two-stage repair. However, neither approach provides a satisfactory outcome. We report a case of patient who underwent two-stage repair, wherein arterial switch operation combined with aortic arch reconstruction was preceded by bilateral pulmonary artery banding; this yielded good outcomes. This approach safely avoids primary repair in the neonatal period and allows for the opportunity to evaluate right ventricle outlet tract stenosis before the definitive repair.

Objectives. The Norwood procedure with a right ventricular-pulmonary artery (RV-PA) shunt for hypoplastic left heart syndrome (HLHS) has been associated with improved postoperative hemodynamics and outcome. This study aimed to evaluate the effectiveness of the Norwood procedure with an RV-PA shunt, and to compare the effect of the Norwood with an RV-PA shunt to the bidirectional Glenn anastomosis (BDG) and the total cavopulmonary connection (TCPC). Methods. This is a retrospective chart review. Between January 2004 and July 2021, 36 patients with HLHS and its variants underwent BDG: 15 patients (6.5 ± 2.3 months) underwent Norwood with an RV-PA shunt (group S); 21 patients (4.0 ± 4.1 months) underwent Norwood with BDG (group G). Nine group S (24.5 ± 6.6 months) and 17 group G (41.0 ± 15.0 months) patients underwent TCPC. Results. Post-BDG pressure in the superior vena cava (SVC) was significantly lower in group S (13 ± 2 mmHg) than in G (18 ± 3 mmHg) (p < 0.01). Three patients in group S and 19 in group G underwent catheter intervention for pulmonary artery within 30 days after BDG (p < 0.01). The percentage of right ventricular end-diastolic volumewas significantly different (group S, 142 ± 41%; group G, 91 ± 28% (p < 0.01)). Conclusions. The Norwood procedure with an RV-PA shunt enabled maintenance of low pressure in SVC and avoidance of percutaneous intervention following BDG. We recommend monitoring the changes in myocardial function post-TCPC.

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