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Open in new tab Download slide OBJECTIVES The aim of this study was to assess the early outcomes and risk factors of paediatric patients requiring extracorporeal membrane oxygenation after cardiac surgery (post-cardiotomy). METHODS Retrospective binational cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age who underwent a paediatric cardiac surgical procedure from 1 January 2013 to 31 December 2021 and required post-cardiotomy extracorporeal membrane oxygenation (PC-ECMO) in the same hospital admission were included in the study. RESULTS Of the 12 290 patients included in the study, 376 patients required post-cardiotomy ECMO (3%). Amongst these patients, hospital mortality was 35.6% and two-thirds of patients experienced a major complication. Hypoplastic left heart syndrome was the most common diagnosis (17%). The Norwood procedure and modified Blalock–Taussig shunts had the highest incidence of requiring PC-ECMO (odds ratio of 10 and 6.8 respectively). Predictors of hospital mortality after PC-ECMO included single-ventricle physiology, intracranial haemorrhage and chylothorax. CONCLUSIONS In the current era, one-third of patients who required PC-ECMO after paediatric cardiac surgery in Australia and New Zealand did not survive to hospital discharge. The Norwood procedure and isolated modified Blalock–Taussig shunt had the highest incidence of requiring PC-ECMO. Patients undergoing the Norwood procedure had the highest mortality (48%). Two-thirds of patients on PC-ECMO developed a major complication. [ABSTRACT FROM AUTHOR]

Background: Analysis of multi‐institutional data and benchmarking is an accepted accreditation standard in cardiac surgery. Such a database does not exist for congenital cardiac surgery in Australia and New Zealand (ANZ). To fill this gap, the ANZ Congenital Outcomes Registry for Surgery (ANZCORS) was established in 2017. Methods: Inclusion criteria included all cardiothoracic and extracorporeal membrane oxygenation (ECMO) procedures performed at five participating centres. Data was collected by data managers, validated by the surgical team, and securely transmitted to a central repository. Results: Between 2015 and 2019, 9723 procedures were performed in 7003 patients. Cardiopulmonary bypass was utilized for 59% and 9% were ECMO procedures. Fifty‐seven percent (n = 5531) of the procedures were performed in children younger than 1 year of age. Twenty‐four percent of procedures (n = 2365) were performed in neonates (≤28 days) and 33% (n = 3166) were performed in children aged 29 days to 1 year (infants). The 30‐day mortality for cardiac cases (n = 6572) was 1.3% and there was no statistical difference between the participating centres (P = 0.491). Sixty‐nine percent of cases had no major post‐operative complications (5121/7456). For cardiopulmonary bypass procedures (n = 5774), median stay in intensive care and hospital was 2 days (IQR 1, 4) and 9 days (IQR 5, 18), respectively. Conclusion: ANZCORS has facilitated pooled data analysis for paediatric cardiac surgery across ANZ for the first time. Overall mortality was low. Non‐risk‐adjusted 30‐day mortality for individual procedures was similar in all units. The continued evaluation of surgical outcomes through ANZCORS will drive quality assessment in paediatric cardiac surgery across ANZ. [ABSTRACT FROM AUTHOR]

Objective: Patients with Fontan physiology may eventually require heart transplantation (HT). We determined the rates and outcomes of HT in a national, population-based multicentre study.Methods: From 1990 to 2015, 1369 patients underwent the Fontan procedure as recorded in the Australia and New Zealand Fontan Registry. We identified those who underwent HT and analysed their outcomes. We compared rates of HT between two catchment areas. In area 1 (n=721), patients were referred to the national paediatric HT programme or its associated adult programme. In area 2 (n=648), patients were referred to the national paediatric HT programme or one of the other adult HT programmes.Results: Mean follow-up time post-Fontan was 11±8 years. Freedom from Fontan failure was 74%±3.9% at 20 years. HT was performed in 34 patients. Patients living in area 1 were more likely to have HT (4.0%, 29/721 vs 0.8%, 5/648, p<0.001) with a cumulative proportion of 3.4% vs 0.7% at 10 years and 6.8% vs 1.2% at 20 years (p=0.002). Area 1 patients were more likely to undergo HT (hazard ratio 4.7, 95% CI 1.7 to 13.5, p=0.003) on multivariable regression. Post-HT survival at 1, 5 and 10 years was 91%, 78% and 71%, respectively. Compared with other patients with congenital heart disease (n=87), Fontan patients had similar in-hospital outcomes and long-term survival.Conclusions: Although HT after the Fontan procedure can be achieved with excellent outcomes, most patients with Fontan failure do not undergo HT. Significant regional differences in rates of HT in Fontan patients exist. [ABSTRACT FROM AUTHOR]

Background: Atrioventricular valve (AVV) regurgitation is increasingly prevalent in patients with a Fontan circulation.Objectives: We sought to determine the impact of ventricular dominance and AVV operation on outcomes in patients with a Fontan circulation and ≥moderate AVV regurgitation.Methods: We conducted a retrospective study, including propensity score matching analysis, of 1,703 patients who survived Fontan operation in Australia and New Zealand from 1987 to 2021.Results: Patients undergoing AVV operation were more likely to have right ventricular (RV) dominance or an atrioventricular septal defect. In the entire cohort, death or transplantation after Fontan operation was significantly higher in patients who underwent AVV operation before or at Fontan completion compared with those who did not (20 years: 18%; 95% CI: 8%-26% vs 13%; 95% CI: 10%-15%; P = 0.03). After propensity score matching, including for RV dominance, there was no significant difference in death or transplantation between the groups (20 years: 18%; 95% CI: 8%-26% vs 16%; 95% CI: 10%-22%; P = 0.41). Only patients with RV dominance who developed ≥moderate AVV regurgitation after Fontan operation were at increased risk of death or transplantation (HR: 2.8; 95% CI: 1.4-5.3; P < 0.01). In patients with left ventricular dominance, there was no significant difference in death or transplantation between patients with ≥moderate AVV regurgitation compared with those with Conclusions: RV dominance, but not AVV surgery itself, was associated with poor outcomes. Moderate or greater AVV regurgitation after Fontan operation is associated with a significantly increased risk of death or transplantation, only in patients with RV dominance. [ABSTRACT FROM AUTHOR]

Background: Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD., Methods: Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 were identified (n = 54) from a binational Fontan registry., Results: Thirty-two patients (60%) underwent repair of TAPVD at a median age of 0.8 (interquartile range: 0.3-1.6) years. Thirty-seven patients (69%) had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late deaths and 3 patients required cardiac transplantation for a failing Fontan circulation. On univariate analysis, the concomitant diagnosis of pulmonary stenosis and right ventricular dominance was associated with late death or transplantation (P = .04). Freedom from late death or transplantation at 15 years after the Fontan operation was 88% ± 7% (95% confidence interval [CI], 67%-96%) for the repaired TAPVD group and 90% ± 6% (95% CI, 67%-98%) for the unrepaired TAPVD group (P = .47). Median follow-up after the Fontan procedure was 10.8 (interquartile range, 6.7-16.2) years. The majority of survivors (94%) were in New York Heart Association functional class I or II. The 15-year freedom from death or transplantation was similar for patients with TAPVD (89% ± 5%; 95% CI, 76%-95%) compared with patients without TAPVD in the Fontan registry (n = 1446; 92% ± 1%; 95% CI, 90%-93%) (P = .12)., Conclusions: Long-term survival of patients with TAPVD who undergo the Fontan operation and survived to hospital discharge is comparable to Fontan survivors without TAPVD., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)