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Reparative operations for interrupted aortic arch with ventricular septal defect
Insights
Primary repair of interrupted aortic arch (IAA) with ventricular septal defect (VSD) in infants shows improved outcomes. Awareness of potential complications like subaortic stenosis is crucial for successful management.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Defects
Background:
- Interrupted aortic arch (IAA) with ventricular septal defect (VSD) is a critical congenital heart condition.
- Surgical repair strategies have evolved to improve outcomes in affected neonates.
Purpose of the Study:
- To evaluate the outcomes of primary versus staged surgical repair for IAA with VSD.
- To identify associated conditions and long-term sequelae following repair.
Main Methods:
- Retrospective analysis of 24 infants undergoing IAA with VSD repair between 1975 and 1982.
- Comparison of outcomes between primary repair and staged/palliative approaches.
- Hemodynamic evaluation via catheterization in survivors 1-3 years post-repair.
Main Results:
- Overall mortality rates were 27% early and 13% late for staged repair, and 23% early for primary repair.
- No significant residual VSD or aortic pressure gradients were noted in survivors.
- Six of 14 survivors developed subaortic stenosis, requiring further intervention.
Conclusions:
- Primary repair of IAA with VSD is associated with favorable outcomes and is the preferred approach.
- Subaortic stenosis and hypocalcemia are important associated conditions to monitor post-repair.
- Continuous improvement in surgical results for neonates with IAA is evident.
Abstract:
From January, 1975, through September, 1982, 24 infants underwent primary or staged repair of interrupted aortic arch (IAA) with ventricular septal defect (VSD). Seven patients had IAA type A and 17 patients had type B. Eleven of the patients, median age 5 days, underwent staged operations and 13 infants, median age 6 days, underwent primary repair. Palliation was by tube graft interposition (six), subclavian-aortic anastomosis (three), left carotid-aortic anastomosis (one), or end-to-side aortic anastomosis (one) combined with pulmonary artery banding (eight) or early VSD closure. With palliation, there were three (27%) early deaths among the eleven patients and one (13%) late death among the eight remaining. Delayed repair at 5 days to 14 months (median 7 months) in seven patients incurred three (43%) early and no late deaths. Primary repair in 13 patients consisted of VSD closure combined with graft interposition (12) or end-to-side aortic anastomosis (one), with three (23%) early and no late deaths. Nine of 14 survivors had hemodynamic evaluation by catheterization 1 to 3 years following repair. None had a significant residual VSD or pressure gradients between the ascending and thoracic aorta. Six had subaortic stenosis, two mild (gradient less than 20 mm Hg) and four severe (gradient greater than 50 mm Hg), necessitating operation. Results of operations in neonates with IAA continue to improve. Essential in management is an awareness that subaortic stenosis and hypocalcemia may be accompaniments of this anomaly. Based on these data, we prefer primary repair for IAA with VSD.