Management and outcomes of double aortic arch in 81 patients

Khalfan Alsenaidi, Rebecca Gurofskya, Tara Karamlou, William G. Williams, Brian W. McCrindle*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

135 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)e1336-e1341
Issue number5
Publication statusPublished - Nov 2006
Externally publishedYes


  • Cardiovascular surgery
  • Congenital cardiac disease
  • Double aortic arch
  • Stridor
  • Vascular rings

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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