Outcome after extracorporeal membrane oxygenation therapy in Norwood patients before the bidirectional Glenn operation.

OBJECTIVES: Patients after the Norwood procedure are prone to postoperative instability. Extracorporeal membrane oxygenation (ECMO) can help to overcome short-term organ failure. This retrospective single-centre study examines ECMO weaning, hospital discharge and long-term survival after ECMO therapy between Norwood and bidirectional Glenn palliation as well as risk factors for mortality. METHODS: In our institution, over 450 Norwood procedures have been performed. Since the introduction of ECMO therapy, 306 Norwood operations took place between 2007 and 2022, involving ECMO in 59 cases before bidirectional Glenn. In 48.3% of cases, ECMO was initiated intraoperatively post-Norwood. Patient outcomes were tracked and mortality risk factors were analysed using uni- and multivariable testing. RESULTS: ECMO therapy after Norwood (median duration: 5 days; range 0-17 days) saw 31.0% installed under CPR. Weaning was achieved in 46 children (78.0%), with 55.9% discharged home after a median of 45 (36-66) days. Late death occurred in 3 patients after 27, 234 and 1541 days. Currently, 30 children are in a median 4.8 year (3.4-7.7) follow-up. At the time of inquiry, 1 patient awaits bidirectional Glenn, 6 are at stage II palliation, Fontan was completed in 22 and 1 was lost to follow-up post-Norwood. Risk factor analysis revealed dialysis (P < 0.001), cerebral lesions (P = 0.026), longer ECMO duration (P = 0.002), cardiac indication and lower body weight (P = 0.038) as mortality-increasing factors. The 10-year mortality probability after ECMO therapy was 48.5% (95% CI 36.5-62.9%). CONCLUSIONS: ECMO therapy in critically ill patients after the Norwood operation may significantly improve survival of a patient cohort otherwise forfeited and give the opportunity for successful future-stage operations.

The right age for the en-bloc rotation of the outflow tracts: a single-centre experience.

OBJECTIVES: The en-bloc rotation of the outflow tracts (EBR) enables an anatomic correction of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. The anatomical condition or previous palliative procedures may allow choosing an elective date for the anatomic correction. The aim of this study was to evaluate the optimal age for performing the EBR based on the largest series published so far. METHODS: At the Children's Heart Center Linz, the EBR was performed in 33 patients between 2003 and 2021. Median age at operation was 74 [interquartile range (IQR) 17-627] days. Twelve patients were newborns (≤28 days), 9 older than 369 days. These 2 groups were compared to the remaining patients each regarding peri- and postoperative data, complications, reinterventions and mortality. The median follow-up period was 5.4 (IQR 0.99-11.74) years. RESULTS: In-hospital mortality was 6.1%. All-cause mortality was lower in patients younger than 369 days at the time of EBR (4.2% vs 44.4% in patients >369 days old, P = 0.013). In newborns, intensive care unit stay (median 18.5 days vs 8 days, P = 0.008) and in-hospital stay (median 29.5 days vs 15 days, P = 0.026) were significantly longer, the risk for postoperative AV block was higher (33.3 vs 0%, P = 0.012) than in patients who had been corrected anatomically after the neonatal period. CONCLUSIONS: The results of this study suggest the deferral of the EBR to the post-newborn period. A significantly higher mortality rate in patients of older age at operation seems to recommend the anatomic correction during the first year of life.

Long-term follow-up after simultaneous arterial switch operation and aortic arch repair.

OBJECTIVES: Taussig-Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. METHODS: At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5-9] days, weight 3.38 (IQR 2.9-3.8) kg and follow-up 9.3 (IQR 3.1-14.5) years. The AA reconstruction was performed without patch material in 49 cases. RESULTS: There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. CONCLUSIONS: The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate.

Surgical and interventional rescue strategies for Fontan failure.

OBJECTIVES: Fontan patients are at lifelong risk for developing complications, which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long-term survival of extracardiac Fontan patients in the modern era was investigated. The objective of this study was to investigate if surgical and interventional procedures in patients with protein-losing enteropathy (PLE) and/or plastic bronchitis (PB) and a failing Fontan circulation can postpone or avoid HTX. METHODS: Retrospective data collection of all children who underwent a Fontan procedure between January 1999 and July 2021 at our centre was performed. Patients were surveyed regarding the occurrence of PLE or PB and their outcome was reported descriptively. HTX-free survival of patients who underwent a rescue procedure due to PLE/PB was evaluated. RESULTS: Three hundred and seventy [94.1% (95% confidence interval, 91.4-96.3)] Fontan patients were free of HTX or death at last follow-up after a median follow-up time of 6.7 years. PB/PLE was diagnosed in 34 patients during the observation period. A rescue procedure was undertaken in 16 pts. at a median time of 6.5 months (range: 1 day to 9.4 years) since the initial diagnosis of PLE/PB. In these patients, HTX-free survival was 75% (95% confidence interval, 47.6-92.7) at a median follow-up time of 4.0 years after the procedure. Range: 3.5 months to 13.9 years. CONCLUSIONS: Extracardiac Fontan patients in the modern era expect reasonable HTX-free survival rates. Surgical and/or interventional rescue strategies for Fontan failure can postpone HTX for a sustained period of time.