부산대학교 도서관

OBJECTIVE:: Treatment of infants with functional single-ventricle and heterotaxy syndrome is challenging due to multistage palliation with high operative mortality and morbidity. We report our experience. METHODS:: Between 1996 and 2017, 20 patients with heterotaxy syndrome underwent their first palliative surgery and form the study population. All relevant preoperative, operative and follow-up data were analyzed retrospectively. RESULTS:: Fourteen (70%) patients required neonatal palliation including a modified Blalock-Taussig shunt (n = 11; 55%), Norwood procedure (n = 1; 5%) or pulmonary artery banding (n = 2; 10%); two patients underwent TAPVC repair during first-stage palliative operation. In the remaining 6 patients (30%), primary bidirectional cavopulmonary anastomosis (BCPA) was the initial palliative surgery. 13 patients underwent BCPA operation as second-stage palliation, whereas one died in the interstage period. Nowadays, 12 of these patients have already undergone total cavopulmonary connection (TCPC) as a third-stage operation after BCPA. In case of Kawashima operation, median time interval before TCPC was 7 years (min 4 years; max 8 years).Mean follow-up time was 9,6 ± 7,9 years. One patient died during long-term follow-up after TCPC. Overall survival for the entire cohort following first surgery were 94,4% at 1 and 5 years and 82,6% at 10 years. CONCLUSIONS:: Despite recent advances, the management of infants with heterotaxy syndrome with single-ventricle physiology is still a challenge for pediatric cardiac surgeons with high described morbidity and mortality. Conversely, in our experience overall mortality is inferior compared to other single-center reports and these patients may expect satisfying mid-to-long term survival. Further studies are needed to assess long-term morbidity.