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[Interrupted aortic arch. A series of 15 patients]
J L De Brux1, J B Subayi, U Hvass
1Service de chirurgie thoracique et cardiovasculaire, Hôpital Bichat, Paris.
Insights
Surgical repair of interrupted aortic arch in infants showed that two-stage repairs yielded poor results, primarily due to issues with aortic arch reconstruction. Harmonious growth of the neo-aortic arch was crucial for long-term survival in these complex congenital heart cases.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Defects
- Thoracic Surgery
Context:
- Interrupted aortic arch (IAA) is a severe congenital heart defect requiring early surgical intervention.
- This study focuses on Celoria and Patton type B IAA, often associated with ventricular septal defects and patent ductus arteriosus.
- Associated anomalies included retro-esophageal right subclavian artery, subaortic stenosis, and right-sided descending aorta.
Purpose:
- To evaluate the outcomes of surgical repair for interrupted aortic arch (IAA) in neonates and infants.
- To assess the effectiveness of one-stage versus two-stage repair strategies.
- To identify factors influencing survival and long-term growth of the neo-aortic arch.
Summary:
- Fifteen infants with Celoria and Patton type B IAA underwent surgical repair between 1983 and 1989.
- A one-stage repair was performed in two cases with severe ascending aorta hypoplasia, resulting in two deaths.
- A two-stage repair was used for other cases, with varying arch reconstruction techniques and universal pulmonary artery banding; seven survived beyond 30 days, with six undergoing complete repair.
- Survival was contingent on harmonious growth of the neo-aortic arch.
Impact:
- A two-stage repair approach for IAA in this series demonstrated disappointing results, largely attributed to suboptimal aortic arch reconstruction via thoracotomy.
- The findings highlight the critical importance of achieving adequate and harmonious growth of the neo-aortic arch for successful surgical outcomes.
- This study underscores the challenges in managing complex IAA and suggests a need for improved reconstructive techniques.
Abstract:
Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.