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Outcomes of Interrupted Aortic Arch Repair in Children With Biventricular Circulation
Eleonora I Andrianova1, Phillip S Naimo2, Tyson A Fricke2
1Department of Paediatrics, University of Melbourne, Melbourne, Australia; Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia.
Insights
Surgical repair of interrupted aortic arch in patients with biventricular circulation shows improved survival for simple cases, but complex cases still have high mortality. Continued advancements are needed for better outcomes in complex congenital cardiac lesions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Research
Background:
- Interrupted aortic arch is a critical congenital heart defect.
- Surgical repair is the standard treatment for interrupted aortic arch.
- Outcomes vary significantly based on associated anomalies.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical repair for interrupted aortic arch in patients with biventricular circulation.
- To compare outcomes between simple and complex cases of interrupted aortic arch.
- To identify factors influencing survival and reoperation rates.
Main Methods:
- Retrospective review of 177 patients undergoing interrupted aortic arch repair (1978-2018).
- Patients categorized into simple (n=122) and complex (n=55) groups based on concomitant anomalies.
- Survival, mortality, and reoperation rates analyzed using competing risks analysis.
Main Results:
- Overall early mortality was 11.9%.
- Between 2000-2018, early mortality was significantly higher in complex (24.1%) vs. simple (3.2%) cases.
- At 15 years, 15.2% required aortic arch reoperation, more common in complex cases (20.0%) than simple cases (9.0%).
Conclusions:
- Survival has improved for simple interrupted aortic arch cases, but remains high for complex congenital cardiac lesions.
- Early mortality in complex cases has decreased but remains a concern.
- Aortic arch reoperation is a significant consideration, particularly in complex cases.
Background:
To evaluate the outcomes after surgical repair of interrupted aortic arch in patients with biventricular circulation.
Methods:
We reviewed all children from a single institution (N = 177) who had interrupted aortic arch repair between 1978 and 2018. Patients were separated into simple (n = 122) and complex (n = 55) group based on their concomitant anomalies.
Results:
Median age at repair was 6 days (range, 1-298 days) and median weight was 3.1 kg (range, 0.95-5.1 kg). Median follow-up time was 11.5 years (mean 12.6 years; range, 0.1-35.9 years). Overall early mortality was 11.9% (21 of 177) and there were 5 late deaths. Era of surgery did not impact on overall survival (P = .37). Between 2000 and 2018, there was a significant difference in early mortality between the simple and complex group (3.2% [2 of 62] vs 24.1% [7 of 29], P = .002). There was an improvement in mortality in the simple group over time (P = .03). Competing risks analysis showed at 15 years after the initial operation 14% had died without arch reoperation, 15.2% had undergone aortic arch reoperation, and 70.8% were alive without arch reoperation. Reoperation on the aortic arch was more common in the complex group compared to the simple group (20.0% [11 of 55] vs 9.0% [11 of 122], P< .001).
Conclusions:
Survival of patients with interrupted aortic arch and associated simple anomalies has improved over time, although mortality in patients with complex congenital cardiac lesions remains high.

