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Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy
R S Sawin1, D G Hall, P B Mansfield
1Department of Surgery, Children's Hospital and Medical Center, Seattle, Washington 98105.
Insights
Interrupted aortic arch with ventricular septal defect has high mortality. Staged surgical repair in infancy offers outcomes comparable to simultaneous repair, improving survival rates for this complex congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Background:
- Interrupted aortic arch (IAA) is a severe congenital heart defect with high mortality.
- Intracardiac lesions, such as ventricular septal defect (VSD), complicate IAA management.
- Early recognition and treatment are critical for survival in IAA patients.
Purpose of the Study:
- To evaluate the outcomes of staged surgical repair for IAA with VSD in infants.
- To compare the results of staged repair with previously reported simultaneous repair strategies.
- To identify factors contributing to improved survival in IAA patients.
Main Methods:
- Retrospective review of nine infants with IAA and VSD treated between 1979 and 1987.
- Primary repair of IAA with pulmonary artery banding in infancy.
- Eventual repair of VSD at a later stage.
- Analysis of operative and overall mortality rates.
Main Results:
- Operative mortality for IAA repair was 11% (1 of 9 patients).
- Overall mortality for the nine patients was 22% after staged VSD repair.
- Staged repair demonstrated outcomes comparable to simultaneous repair approaches.
- Prostaglandin E use and improved perioperative stabilization likely contributed to survival.
Conclusions:
- Staged surgical repair of interrupted aortic arch with ventricular septal defect is a viable option.
- This approach yields results comparable to simultaneous repair in infancy.
- Improved perioperative care, including prostaglandin E, is crucial for better survival in complex congenital heart defects.
Abstract:
Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.