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Surgical management of patients with interrupted aortic arch and severe subaortic stenosis
M N Ilbawi1, F S Idriss, S Y DeLeon
1Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, IL 60614.
Insights
Palliative surgery for interrupted aortic arch with subaortic obstruction is challenging. A pulmonary artery-descending aorta conduit with distal banding offers improved survival in neonates.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Interrupted aortic arch (IAA) is a critical congenital heart defect.
- Severe subaortic stenosis (SAS) associated with IAA presents significant surgical challenges.
- Posterior displacement of the conal septum is a common cause of SAS in IAA.
Purpose of the Study:
- To evaluate the efficacy of a specific palliative surgical approach for IAA with SAS.
- To compare outcomes between different surgical techniques in this high-risk patient group.
Main Methods:
- Retrospective analysis of 10 neonates with IAA and SAS.
- Preoperative echocardiography and cardiac catheterization.
- Surgical intervention involving pulmonary artery banding and aorta-to-descending aorta bypass graft.
Main Results:
- Four patients underwent ascending aorta-descending aorta bypass graft with pulmonary artery banding, resulting in 100% operative mortality.
- Six patients received a pulmonary artery-descending aorta conduit with distal pulmonary artery banding; all survived.
- Long-term follow-up (3 months to 4 years) showed good outcomes for the surviving patients.
Conclusions:
- A pulmonary artery-descending aorta conduit combined with distal pulmonary artery banding is a viable palliative strategy for IAA with severe subaortic stenosis.
- This technique significantly improves survival compared to ascending aorta-descending aorta bypass in this complex condition.
Abstract:
Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.