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

Recent Era Outcomes of Mechanical Circulatory Support in Children With Congenital Heart Disease as a Bridge to Heart Transplantation

The objective of the study is to compare the clinical characteristics, risk factors, and overall survival (waitlist and posttransplant) outcomes in children with congenital heart disease (CHD) bridged to transplantation with either a ventricular assist device (VAD) versus extracorporeal membrane oxygenation (ECMO) versus no mechanical circulatory support (MCS) in the recent era. The study included 2,899 primary heart transplantations in patients \u3c18 years with CHD between 2010 and 2019 from the United Network Organ Sharing database. Patients who had ECMO or VAD at listing or while listed were included, and their waitlist and posttransplant outcomes were compared with CHD patients who did not require MCS. Of all, 464 (16%) had ECMO and 200 (7%) VAD at the time of or during the listing. The VAD utilization increased over the last decade (4% in 2010 to 10% in 2019, p \u3c 0.01). The 90 days post-MCS survival was better with VAD than ECMO (67 vs. 49%, p \u3c 0.01). The transplantability rate at 90 days was decreased with younger age (odds ratio [OR], 0.91; 95% CI, 0.86-0.95), lower body mass index (BMI) (OR, 0.93; 95% CI, 0.89-0.98) and lower albumin \u3c3g/dl (OR, 0.6; 95% CI, 0.53-0.7). The multivariate model predicted that lower BMI (OR, 1.12; 95% CI, 1.06-1.18), pretransplant ECMO (OR, 2.19; 95% CI, 1.39-3.45), and higher bilirubin (OR, 1.15; 95% CI, 0.97-1.36) decreased 1-year posttransplant survival. Patients transplanted with VAD had better 1-year survival than ECMO (88 vs. 70%, p = 0.01). Waiting list survival of children with CHD supported by VAD is better compared to ECMO. The 1-year posttransplantation outcome of CHD patients supported by VAD is similar to the no MCS patients and better than ECMO-supported patients. There is no significant difference in post-HT survival between patients transitioned from ECMO to VAD while listed and those with VAD-first