Double Aortic Arch Clinical Presentation

Updated: Dec 31, 2019
  • Author: Doff B McElhinney, MD; Chief Editor: Stuart Berger, MD  more...
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Presentation of symptoms in patients with double aortic arch depends on several factors, including the severity of tracheal compression, esophageal compression, or both and whether associated anomalies are present. Note the following:

  • Among patients with a vascular ring, those with double aortic arch tend to present earlier than those with other anatomic variations. The classic history in a patient with double aortic arch is noisy breathing noted by the parents during the first few weeks of life.

  • Young patients may have experienced episodes that often are termed apparent life-threatening events (ALTE) or death spells, in which acute apneic or severe obstructive events are accompanied by cyanosis. Patients with less severe tracheal compression may give a history of persistent respiratory symptoms without frank stridor, often treated as asthma or bronchiolitis, or a history of recurrent lower respiratory infections.

  • Esophageal symptoms include emesis, choking, or dysphagia and are more common in older infants and children than in young infants.

  • Occasionally, patients may reach older childhood or adulthood before developing persistent or progressive symptoms of dysphagia, respiratory symptoms, or both.

  • Rarely, fetal echocardiography may reveal double aortic arch and types of vascular ring.

  • In neonates with associated cardiac or noncardiac anomalies, a double aortic arch may be diagnosed incidentally during the course of evaluation.


Physical Examination

Physical findings can vary, often in accordance with the patient's history.

Newborns with associated anomalies may have no evidence of a vascular ring on physical examination, but this situation is the exception because most patients have readily recognizable physical signs.

The classic sign of double aortic arch and of vascular rings in general is nonpositional stridor; however, many young infants with double aortic arch have adventitious expiratory breath sounds, as well as the characteristic inspiratory stridor. Respiratory findings typically do not improve with nebulized bronchodilator therapy and usually are more prominent with agitation or crying.



A persistent double aortic arch occurs when abnormal regression of the embryonic aortic arch segments is present, in which both the left and right aortic arches remain intact. With the different forms of double aortic arch, different segments of the embryonic aortic arch system, which normally regress, remain patent.

Factors responsible for the aberrant persistence of certain aortic arch segments have not been clearly identified, and the pathogenesis of this anomaly remains a mystery. Double aortic arch typically occurs without associated cardiovascular defects, although other lesions may be present, and accordingly, it is not usually found as part of a syndromic complex.

In a study at the author's institution, band 22q11 deletions were found in 3 of 22 patients (14%) with double aortic arch. [4] This chromosomal anomaly is associated with aortic arch anomalies in patients with other forms of conotruncal heart disease and other isolated vascular abnormalities, and band 22q11 deletion is likely to be an important etiologic factor in double aortic arch. Most such mutations arise de novo, and no recognizable inheritance pattern is present.

Familial recurrence of double aortic arch has been reported, supporting a genetic etiology for this anomaly. Teratogen-induced double aortic arch in animal models also has been reported. The mechanisms and significance of these models have not been elucidated.