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Diagnosis:Double Aortic Arch

Discussion: Double aortic arch is a class of vascular ring which often causes symptoms from compression of the trachea and/or esophagus. Aortic arch rings, which include double aortic arch, represent approximately 1-2% of congenital heart disease. Patients often present with inspiratory stridor, expiratory wheezes, and tachypnea usually within the first 6 months of life. Feeding is often poor with choking and dysphagia proportional to degree of obstruction. Patients presenting later than 6 months often have less severe symptoms and tend to have a more favorable natural course. Severe recurrent respiratory infections, cyanosis, apnea, unconsciousness, and even death are complications of severe obstruction. Though diagnosis can often be made with echocardiographic imaging, MR is proving to be of greater utility when the diagnosis is in question or to further define anatomic relationships of surgically important structures. Surgical correction is indicated in symptomatic patients with particular attention to vascular anatomy and potential constricting bands of tissue.

Long-term prognosis is excellent with very low post-op morbidity and mortality in the absence of other heart defects.

References:
1. Higgins CB, Caputo GR. Role of MR Imaging in Acquired and Congenital
Cardiovascular DiseaSe AJR 1993;161:13-22.

2. Vascular Rings and Slings, in Kirklin JW, Bassatt-Boyes BG: Cardiac Surgery,
Morphology, Diagnostic Criteria, Natural History, Techniques, Results and
Indications. New York, John Wiley 1986 pp 1111-1119.

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