Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome

摘要: Abstract Background Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac extracardiac anomalies. We report current era palliation results. Methods Between 2002 2012, 67 underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) examined factors survival. In addition, early late outcomes following first-stage were compared a matched contemporaneous control group patients nonheterotaxy Results Fifty-eight (87%) required neonatal palliation, including modified Blalock-Taussig shunt (n = 34; 51%), Norwood operation (n = 12; 18%) or pulmonary artery band 18%), whereas 9 (13%) primary procedure. analysis showed that at 1 year surgery, 29% the had died undergone transplantation 63% By 5 years procedure, 64% Fontan The overall 8-year survival rate was 66%. On multivariable analysis, mortality unplanned reoperation (hazard ratio [HR], 2.9; 95% confidence interval [CI], 1.1-7.3; P  = .005) total anomalous venous connection repair (HR, 2.3; CI, 1.0-5.6;  = .056). Comparison anomalies in higher in-hospital death (27% 10%;  = .022), significantly longer durations ventilation intensive care unit stay. Interstage mortality, progression procedure comparable 2 groups. Conclusions management remains challenging. First-stage high operative increased resource utilization owing surgical morbidity. Nonetheless, beyond hospital discharge are those for other Efforts improve should focus on perioperative care.