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Outcomes in patients with interrupted aortic arch and ventricular septal defect. A multiinstitutional study.
R A Jonas1, J M Quaegebeur, J W Kirklin
1Department of Cardiac Surgery, Children's Hospital, Boston, Mass.
Insights
Survival after surgical repair for interrupted aortic arch and ventricular septal defect is significantly impacted by patient factors and surgical approach. Identifying risk factors improves outcomes for neonates undergoing this complex cardiac procedure.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Neonatal Intensive Care
Background:
- Interrupted aortic arch (IAA) with ventricular septal defect (VSD) is a critical congenital heart defect.
- Neonates with IAA and VSD present complex surgical challenges.
- Early diagnosis and intervention are crucial for survival.
Purpose of the Study:
- To identify risk factors associated with mortality in neonates undergoing repair for IAA and VSD.
- To evaluate the impact of different surgical strategies on long-term survival.
- To analyze institutional and procedural risk factors.
Main Methods:
- Multi-institutional retrospective study of 183 neonates with IAA and VSD.
- Analysis of pre-operative, intra-operative, and post-operative data.
- Kaplan-Meier survival analysis and risk factor identification.
Main Results:
- Overall survival at 4 years post-repair was 63% among 174 neonates who underwent repair.
- Risk factors for death included low birth weight, younger age, IAA type B, specific VSD types, smaller VSD size, and subaortic narrowing.
- Procedural risks included repair without concomitant procedures for complex obstructions, Damus-Kaye-Stansel anastomosis, and subaortic myectomy.
- One-stage repair with aortic/arch augmentation showed better survival in complex cases.
Conclusions:
- Patient-specific factors like birth weight and age significantly influence survival post-IAA/VSD repair.
- Surgical technique and management of associated left heart obstructions are critical determinants of outcome.
- Institutional experience may also play a role in patient survival.
Abstract:
Among 183 neonates with interrupted aortic arch and ventricular septal defect entering a multiinstitutional study between 1987 and 1992, nine died before repair was accomplished. Among the remaining 174, survival at 1 month and 1, 3, and 4 years after repair was 73%, 65%, 63%, and 63%, respectively. The risk factors for death were low birth weight, younger age at repair, interrupted arch type B, outlet and trabecular ventricular septal defects, smaller size of the ventricular septal defect, and subaortic narrowing. Echocardiographically measured dimensions (expressed as Z-values) at all levels of the left heart-aorta complex were small. Two among thirty institutions were risk factors, and two others possibly were. Procedural risk factors for death after repair were (1) repair without concomitant procedures in patients with other important levels of obstruction in the left heart-aorta complex, (2) a Damus-Kaye-Stansel anastomosis, and (3) subaortic myotomy/myectomy in the face of subaortic narrowing. One-stage repair plus ascending aorta/arch augmentation had the highest predicted time-related survival in the 20% of patients with interrupted aortic arch and one or more coexisting levels of obstruction in the left heart-aorta complex, as did initial repair without or with aorta/arch augmentation in the 80% without these.