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Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome

Published online by Cambridge University Press:  14 September 2015

Daniela Laux ,
Lucile Houyel ,
Fanny Bajolle ,
Francesca Raimondi ,
Younes Boudjemline  and
Damien Bonnet
Daniela Laux
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union Centre de Référence Malformations Cardiaques Congénitales Complexes – M3C, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France, European Union
Lucile Houyel
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes – M3C, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France, European Union
Fanny Bajolle
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Francesca Raimondi
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Younes Boudjemline
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Damien Bonnet*
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
*
Correspondence to: D. Bonnet, MD, PhD, Necker-M3C, Pediatric Cardiology, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France, European Union. Tel: +3 314 449 4344; Fax: +3 314 449 4340; E-mail: damien.bonnet@nck.aphp.fr
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Abstract

Background

Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach.

Objective

To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up.

Methods

A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections.

Results

Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors.

Discussion

Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Keywords

Discordant atrioventricular connectionconcordant ventriculo-arterial connectionatrial switchheterotaxyleft isomerism
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Supplement 1 , January 2016 , pp. 127 - 138
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Copyright
© Cambridge University Press 2015 

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