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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.
Insights
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65% in the initial 5 years to a minimal rate in the latter 5 years, with deaths primarily in non-operated or treatment-withdrawn infants.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.
Abstract:
Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when only eight of the subsequent 29 patients underwent catheterization. Since 1987, all patients underwent surgery after cross-sectional echocardiography. Fifteen infants had a type A IAA and 35 had type B. All had associated cardiac anomalies. Four infants were not operated on. In the initial 5-year period, of 17 infants who were surgically treated, four had a one-stage total repair, the remaining had a two-stage repair with initial reconstruction of the arch and pulmonary artery banding. There was an overall surgical mortality of 65%, reflecting the precarious state of many of these infants before surgery with a significant contribution from unrelieved subaortic stenosis. In the latter 5-year period, 29 underwent surgery, 22 had a one-stage total repair. There were three deaths, all in infants whose active treatment was withdrawn. The outcome of the survivors has generally been good, subsequent surgery being mainly related to the associated anomalies (e.g., recurrent subaortic stenosis, conduit replacement). Over this 10 year period the greater accuracy of noninvasive diagnoses, and perioperative intensive care, have led to an improvement in the preoperative state of these infants. Single-stage total repair is our procedure of choice.