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Tetralogy of Fallot with coarctation of the aorta: a newly recognised developmental and anatomic syndrome

Published online by Cambridge University Press:  26 March 2014

Elodie Perdreau ,
Lucile Houyel  and
Alban-Elouen Baruteau
Elodie Perdreau*
Affiliation:
Service des Cardiopathies Congénitales, Hôpital du Haut-Lévêque, CHU de Bordeaux, Pessac, France
Lucile Houyel
Affiliation:
Service de Chirurgie des Cardiopathies Congénitales, Centre Chirurgical Marie-Lannelongue-M3C, Université Paris-Sud, Le Plessis-Robinson, France
Alban-Elouen Baruteau
Affiliation:
Service de Chirurgie des Cardiopathies Congénitales, Centre Chirurgical Marie-Lannelongue-M3C, Université Paris-Sud, Le Plessis-Robinson, France
*
Correspondence: Dr E. Perdreau, Secrétariat du Pr Thambo, Hôpital du Haut-Lévêque, Avenue de Magellan, 33604 Pessac, France. Tel: +00 33 6 01 49 00 88; Fax: +00 33 1 40 94 88 67; E-mail: elop85@yahoo.fr
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Abstract

Tetralogy of Fallot and coarctation of the aorta is an exceptional association. We report here four cases of infants referred for tetralogy of Fallot with or without pulmonary atresia associated with aortic coarctation from 1974 to 2013. All had a right aortic arch, and the coarctation was abnormally situated between the right common carotid and the right subclavian arteries. In all, two infants had an abnormal left subclavian artery and one child had DiGeorge syndrome. All underwent staged surgical repair of the left and right-sided obstructions. A review of the literature shows two types of coarctation in this context. In left aortic arch, coarctation is situated distal to the left subclavian artery. In right aortic arch, coarctation is distal to the right common carotid artery, mirror-image of interrupted left aortic arch type B, associated with anomalies of the branches of the aorta, and should be considered a complex anomaly of aortic arches in the setting of an outflow tract defect due to abnormal migration of cardiac neural crest cells. Screening for this unusual association is critical in the initial assessment of all patients with tetralogy of Fallot.

Keywords

Tetralogy of Fallotcoarctation of the aortaright aortic archcardiac neural crest cells migration
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 4 , August 2014 , pp. 714 - 720
Copyright
© Cambridge University Press 2014 

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