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Interrupted aortic arch in infancy: a 10-year experience.
S Menahem1, A U Rahayoe, W J Brawn
1Department of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Pediatric Cardiology
|October 1, 1992
Summary
Interrupted aortic arch (IAA) in infants often presents with severe cardiac issues. Advances in diagnosis and care have improved outcomes, with single-stage total repair becoming the preferred surgical approach.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Neonatal Critical Care
Background:
- Interrupted aortic arch (IAA) is a severe congenital heart defect often presenting in neonates with critical cardiac failure or shock.
- Historically, diagnostic approaches for IAA involved invasive cardiac catheterization, contributing to delayed or suboptimal surgical management.
- Associated cardiac anomalies are common in infants with IAA, complicating surgical outcomes.
Purpose of the Study:
- To review the surgical outcomes of infants with interrupted aortic arch (IAA) over a 10-year period.
- To evaluate the impact of diagnostic advancements and evolving surgical strategies on IAA patient outcomes.
- To identify the preferred surgical approach for IAA based on the study's findings.
Main Methods:
- Retrospective review of 50 infants diagnosed with IAA between 1979 and 1988.
- Comparison of diagnostic methods (cardiac catheterization vs. echocardiography) and surgical repair strategies (one-stage vs. two-stage) between two 5-year periods.
- Analysis of overall surgical mortality, causes of death, and long-term outcomes of survivors.
Main Results:
- A significant decrease in the use of cardiac catheterization and an increase in one-stage total repairs were observed over the study period.
- Overall surgical mortality decreased from 65% in the initial 5 years to a minimal rate in the latter 5 years, with deaths primarily in non-operated or treatment-withdrawn infants.
- Survivors generally had good outcomes, with subsequent surgeries mainly addressing associated anomalies.
Conclusions:
- Improved noninvasive diagnostic accuracy (echocardiography) and enhanced perioperative intensive care have significantly improved the preoperative condition and surgical outcomes of infants with IAA.
- Single-stage total repair has emerged as the preferred surgical procedure for interrupted aortic arch.
- The study highlights a successful transition towards less invasive diagnostics and more effective surgical management for IAA.