Older children at time of norwood operation have ongoing mortality vulnerability that continues after the cavopulmonary connection

BackgroundDelayed 1st stage palliation of children with hypoplastic left heart syndrome (HLHS) and related pathologies can be associated with poor outcomes due to development of progressive pulmonary vascular disease) and volume load effects on systemic ventricle and atrioventricular valve (AVV). We examine current era survival of this patients’ subgroup.MethodsFifty-five infants >2weeks old underwent Norwood surgery (2003-07). Separate competing risks analyses were performed to model outcomes (death, transition to next stage) after Norwood and after bidirectional cavo-pulmonary connection (BCPC).ResultsMedian age was 32 days (range:15-118). 47% had HLHS, 53% had other complex univentricular variants. Mean ascending aorta was 4.4±1.9mm, 10% had impaired ventricle function, 11% moderate AVV regurgitation, 32% restrictive pulmonary venous return. Pulmonary blood flow was established via aortopulmonary shunt (30) or Sano shunt (25) Following Norwood, patients required longer ventilation, more oxygen & nitric oxide and had higher inotropic scores compared to traditional management protocol. Competing risks analysis showed that 2-years after Norwood 39% had died and 57% underwent BCPC. 4-years after BCPC, 15% had died and 85% underwent Fontan operation. Overall 3 years survival post-Norwood was 53%. Factors associated with mortality were age, lower weight at Norwood, impaired ventricular function, longer circulatory arrest and lower pre-BCPC saturation.ConclusionsChildren >2weeks old undergoing Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multi-stage palliation. Elevated pulmonary vascular resistance, volume load effects such as systemic ventricle impairment and AVV regurgitation are commonly evident in patients who fail or don’t qualify to proceed to next stage palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated