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[Surgical treatment of interrupted aortic arch in infants under three months of age]
Insights
Surgical repair of interrupted aortic arch (IAA) in infants is feasible. Staged operations for IAA with VSD offer an 86% survival rate, while primary repair benefits infants with IAA and significant LVOTO.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Defects
- Vascular Surgery
Context:
- Interrupted aortic arch (IAA) is a severe congenital heart defect requiring timely surgical intervention.
- Infants with IAA often present with complex anatomy, including ventricular septal defects (VSD) or left ventricular outflow tract obstruction (LVOTO).
- Surgical strategies have evolved to improve outcomes for these critically ill neonates.
Purpose:
- To evaluate the surgical outcomes of infants with interrupted aortic arch (IAA) treated between 1982 and 1988.
- To assess the efficacy of staged surgical repair versus primary repair for different IAA subtypes and associated anomalies.
- To identify optimal surgical approaches for IAA based on associated cardiac defects.
Summary:
- Eight infants under 3 months underwent surgical treatment for IAA (5 Type A, 3 Type B).
- Seven patients with VSD had staged operations, achieving an 86% survival rate (6/7 survivors).
- One infant with aortopulmonary window underwent primary repair; operative death occurred in a complex case with subaortic stenosis requiring modified Damus-Kaye-Stansel procedure.
Impact:
- Reliable aortic arch reconstruction and pulmonary artery banding are achievable in critically ill infants with IAA and VSD.
- Primary repair offers a better survival chance for infants with IAA associated with significant LVOTO, truncus arteriosus, or aortopulmonary window.
- Long-term follow-up shows survivors are doing well, with no significant aortic pressure gradients.
Abstract:
From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.