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.