Published online September 25, 2006
PEDIATRICS Vol. 118 No. 5 November 2006, pp. e1336-e1341 (doi:10.1542/peds.2006-1097)

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ARTICLE

Management and Outcomes of Double Aortic Arch in 81 Patients

Khalfan Alsenaidi, MD^a, Rebecca Gurofsky^a, Tara Karamlou, MD^b, William G. Williams, MD^b and Brian W. McCrindle, MD, MPH^a

^a Division of Cardiology, Department of Pediatrics
^b Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada

OBJECTIVES. Double aortic arch is a common form of complete vascular ring, encircling both the trachea and esophagus, resulting in noncardiac morbidity. We sought to describe management and outcomes in a large single institution clinical series.

PATIENTS AND METHODS. We reviewed the medical charts of all of the patients diagnosed with double aortic arch at age <18 years. Data regarding clinical presentation, anatomy, management, and outcomes were abstracted.

RESULTS. We identified 81 patients (67% males) born between 1964 and 2005 and presenting at a median age of 5 months (range: birth to 10.3 years). Respiratory symptoms were present in 91%, including stridor in 77%. Gastrointestinal symptoms were present in 40%, with choking with feeds being most common. The dominant branch of the double aortic arch was right in 72%. Associated cardiac anomalies were present in 18%, with noncardiac anomalies in 7%. Repair was performed in 79 patients at a median age of 6 months. There were 2 deaths after surgical repair with no late deaths, with Kaplan-Meier survival estimates of 96% at 5 years. Postoperative complications included chylothorax in 9%. Only 1 patient required reoperation. The most common symptoms at most recent follow-up were respiratory (54%) followed by gastrointestinal symptoms (6%). Postoperative tracheal stenosis was documented in 14%, with tracheomalacia in 7%. There were no late reoperations and no evidence of aortic arch obstruction.

CONCLUSIONS. Outcomes are excellent after repair of double aortic arch, although persistent respiratory symptoms are frequent and probably associated with previous compression-related maldevelopment of the trachea and major airways.

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Key Words: double aortic arch • vascular rings • stridor • congenital cardiac disease • cardiovascular surgery

Abbreviations: DAA—double aortic arch

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Accepted Jun 5, 2006.

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