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Aortic atresia and interrupted aortic arch communicating through external carotid anastomosis

Published online by Cambridge University Press:  24 May 2019

Volodia Dangouloff-Ros Open the ORCID record for Volodia Dangouloff-Ros [Opens in a new window] ,
Nathalie Boddaert  and
Francesca Raimondi
Volodia Dangouloff-Ros*
Affiliation:
Department of Pediatric Radiology, Hôpital Universitaire Necker Enfants Malades, AP-HP, 149 rue de Sèvres, 75105 Paris, France INSERM U1000, 149 rue de Sèvres, 75015 Paris, France University René Descartes, PRES Sorbonne Paris Cité, 12 rue de l’Ecole de Médecine, Paris, France UMR 1163, Institut Imagine, 24 boulevard du Montparnasse, 75015 Paris, France
Nathalie Boddaert
Affiliation:
Department of Pediatric Radiology, Hôpital Universitaire Necker Enfants Malades, AP-HP, 149 rue de Sèvres, 75105 Paris, France INSERM U1000, 149 rue de Sèvres, 75015 Paris, France University René Descartes, PRES Sorbonne Paris Cité, 12 rue de l’Ecole de Médecine, Paris, France UMR 1163, Institut Imagine, 24 boulevard du Montparnasse, 75015 Paris, France
Francesca Raimondi
Affiliation:
Department of Pediatric Radiology, Hôpital Universitaire Necker Enfants Malades, AP-HP, 149 rue de Sèvres, 75105 Paris, France Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes – M3C Hôpital Universitaire Necker Enfants Malades, AP-HP, 149 rue de Sèvres, 75105 Paris, France
*
Author for correspondence: Dr Volodia Dangouloff-Ros, Assistance-Publique Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Department of Pediatric Radiology, 149 rue de Sèvres, 75015 Paris, France. Tel: +33-1-44496719; Fax: +33-1-44495170; E-mail: volodia.dangouloff-ros@aphp.fr
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Abstract

We describe the case of a newborn girl who displayed association of aortic atresia and interrupted aortic arch, with retrograde flow in ascending aorta, through extracranial anastomoses between vertebral arteries (arisen from descending aorta) and external carotids.

Keywords

Aortaheart defectscongenitalcarotid arteriesCT
Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 29 , Issue 5 , May 2019 , pp. 699 - 700
Copyright
© Cambridge University Press 2019 

Association of aortic atresia and interrupted aortic arch is usually lethal due to coronary hypoperfusion, unless coronary flow is provided by anastomosis with pulmonary vesselsReference Careddu, Oppido, Ialonardi and Gargiulo 1 – Reference Yew, Coleman and Calder 4 . A girl had prenatal diagnosis of conoventricular ventricular septal defect, aortic atresia, and probable aortic arch interruption. Neonatal echocardiography confirmed prenatal diagnosis, with large ductus arteriosus vascularising descending aorta. During the first day of life, she presented myocardial ischemia, with troponin elevation at 3884 ng/L.

Cervico-thoracic angio-CT scanner displayed aortic valve atresia with hypoplastic ascending aorta connected to common carotid arteries. Aortic arch was interrupted after carotid arteries. Left-sided descending aorta was connected to a large pulmonary trunk through a large ductus arteriosus. Right and left subclavian arteries arise from descending aorta, right subclavian artery having retro-esophageal course, without Kommerell diverticulum. Large right and left vertebral arteries arise from subclavian arteries. On both sides, they vascularised large collateral arteries arising from the junction of their V3 and V4 segments, which were connected to the external carotid arteries. Therefore, coronary arteries were supplied by retrograde flow in common carotids arteries coming from subclavian arteries through an anastomosis of vertebral arteries and external carotid arteries (Fig 1).

Figure 1. 3D reconstruction of arterial system. Anterior (left image), right (middle image), and posterior (right image) view of aortic, supra-aortic, and pulmonary arteries. In red, hypoplastic ascending aorta is connected to common carotid arteries, which terminates as external carotid arteries without internal carotids arteries. In blue, pulmonary artery is connected with descending aorta through a large patent ductus arteriosus; descending aorta is connected with subclavian arteries which continue as two large vertebral arteries. Anastomoses between these two systems are located on both sides at the junction of V3 and V4 segments of vertebral arteries, allowing a retrograde flow within carotid arteries and ascending aorta.

At 7 days of age, the patient underwent surgical repair, with pulmonary homograft and Damus–Kaye–Stansel procedure to reconstruct aortic arch, surgical connection of right ventricle to pulmonary arteries using a prosthetic conduit.

We report here the first case of bilateral extracranial anastomosis between distal vertebral arteries and external carotid arteries, providing retrograde flow to carotids arteries, ascending aorta, and finally to coronary arteries.

Author ORCIDs

Volodia Dangouloff-Ros 0000-0003-1260-5886

Acknowledgements

None.

Financial Support

This research received no specific grant from any funding agency, commercial or not-for-profit sectors.

Conflict of Interest

None.

Ethical Standards

The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the institutional committees.

References

Careddu, L, Oppido, G, Ialonardi, M, Gargiulo, G. Aortic atresia with interrupted aortic arch: a combination incompatible with life? Interact Cardiovasc Thorac Surg 2015; 21: 272–273.CrossRefGoogle ScholarPubMed
Tannous, HJ, Moulick, AN, Jonas, RA. Interrupted aortic arch and aortic atresia with circle of Willis-dependent coronary perfusion. Ann Thorac Surg 2006; 82: e11–e13.CrossRefGoogle ScholarPubMed
Maruo, A, Oshima, Y, Hasegawa, T. Left subclavian artery perfusion during the Norwood operation for interrupted aortic arch with aortic atresia. Ann Thorac Surg 2016; 101: 764–766.CrossRefGoogle ScholarPubMed
Yew, G, Coleman, D, Calder, L. Aortic valvar atresia, interrupted aortic arch, and quadricuspid pulmonary valve: a rare combination. Pediatr Cardiol 2005; 26: 455–459.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1. 3D reconstruction of arterial system. Anterior (left image), right (middle image), and posterior (right image) view of aortic, supra-aortic, and pulmonary arteries. In red, hypoplastic ascending aorta is connected to common carotid arteries, which terminates as external carotid arteries without internal carotids arteries. In blue, pulmonary artery is connected with descending aorta through a large patent ductus arteriosus; descending aorta is connected with subclavian arteries which continue as two large vertebral arteries. Anastomoses between these two systems are located on both sides at the junction of V3 and V4 segments of vertebral arteries, allowing a retrograde flow within carotid arteries and ascending aorta.