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Long-term Outcomes After Interrupted Aortic Arch Repair
J Cole Miller1, Romie N Velani2, Wade D Miller3
1Children's Healthcare of Atlanta Cardiology, Atlanta, Georgia; Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia.
The Annals of Thoracic Surgery
|February 15, 2024
Summary
Long-term survival after interrupted aortic arch (IAA) repair is over 80% at 30 years. However, staged repair and left ventricular outflow tract obstruction interventions increase mortality risk in infants.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Clinical Outcomes Research
Background:
- Interrupted aortic arch (IAA) is a severe congenital heart defect often associated with left ventricular outflow tract obstruction (LVOTO) and DiGeorge syndrome.
- Infantile surgical repair of IAA is high-risk, with limited data on long-term survival.
- Understanding factors influencing long-term outcomes is crucial for improving patient management.
Purpose of the Study:
- To assess long-term survival outcomes after interrupted aortic arch (IAA) repair.
- To identify patient characteristics and surgical approaches associated with mortality.
- To evaluate trends in survival over time.
Main Methods:
- Retrospective cohort study using the Pediatric Cardiac Care Consortium registry (1982-2003).
- Kaplan-Meier plots and Cox proportional hazards regression for survival analysis.
- Matching with the US National Death Index for post-discharge mortality tracking.
Main Results:
- Of 390 patients, 79.2% survived to discharge; 30-year survival reached 80.7% for hospital survivors.
- Type B IAA, staged repair, and initial LVOTO interventions were associated with increased mortality risk.
- No significant association found for LVOTO without intervention or DiGeorge syndrome presence.
Conclusions:
- Staged repair, type B IAA, and initial LVOTO interventions are high-risk factors for 30-year mortality.
- Survival outcomes show an improving trend over the study period.
- Minimizing staged approaches and LVOTO-related risks is essential for improving long-term survival.
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