Double Aortic Arch with TOF: Single Stage Single Incision Approach | Insight Medical Publishing

Introduction: Double Aortic Arch (DAA) is a rare accompaniment of Tetralogy of Fallot (TOF). Traditionally DAA surgery is accomplished through a posterolateral thoracotomy, which may be combined with a median sternotomy for total correction of TOF either in the same setting or as a staged procedure. In this case report we present the more economical single stage approach via median sternotomy. Background: Double aortic arch is the most common complete vascular ring and comprises 1% of operable congenital cardiac disease. The most common cyanotic congenital heart lesions associated with DAA are TOF and transposition of great arteries. Literature on the approach to DAA with TOF is confusing as some propound a staged approach via postero-lateral thoracotomy and subsequent sternotomy, some a single stage approach using both median sternotomy and postero-lateral thoracotomy, while we agree with Zhou Dan et al. in approaching via median sternotomy as a single stage approach to correct both DAA and TOF. Case: A 3-year-old presented with a diagnoses of DAA with TOF, confirmed by relevant imaging. Median sternotomy was used and the non-dominant left arch distal to left subclavian artery was divided before intracardiac repair for TOF. Conclusion: The difficulty in handling the non-dominant arch from the midline accounts for the prevalence of the two incision approach wherein the arch is tackled by a separate posterolateral thoracotomy. This case report describes successful management of TOF with DAA via median sternotomy, where we used an interesting technique to successfully clamp and divide the difficult to access non-dominant arch.