PMID: 3663429Sep 1, 1987Paper

Interrupted right aortic arch in DiGeorge syndrome

British Heart Journal
P MoermanL G Van der Hauwaert

Abstract

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.

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Citations

May 13, 1999·The Annals of Thoracic Surgery·D B McElhinneyA M Rudolph
Apr 1, 1994·Hospital Topics·G van ServellenB Leake
Aug 9, 2007·Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography·Ming-Ming Sim, Chein-Chung Chen
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Apr 14, 2018·Seminars in Cardiothoracic and Vascular Anesthesia·Kevin Friedman
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