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Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation

Published online by Cambridge University Press:  24 September 2014

Christopher J. Knott-Craig ,
Thittamaranahalli Kariyappa S. Kumar ,
Alejandro R. Arevalo  and
Vijaya M. Joshi
Christopher J. Knott-Craig*
Affiliation:
Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
Thittamaranahalli Kariyappa S. Kumar
Affiliation:
Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
Vijaya M. Joshi
Affiliation:
Divison of Pediatric Cardiology, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
*
Corresponding to: Dr C. J. Knott-Craig, Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, 50N Dunlap, Memphis, TN 38103, United States of America. Tel: +901 2875995; E-mail: cknottcr@uthsc.edu
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Abstract

Objective: Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. Methods: A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Results: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Conclusions: Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Keywords

neonatepulmonary valvetricuspid valvesurgical techniquesright ventricle
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 6 , August 2015 , pp. 1119 - 1123
Copyright
© Cambridge University Press 2014 

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Footnotes

Meeting Presentation: The Society of Thoracic Surgeons 50th Annual Meeting, 2014, Orlando, Florida, United States of America.

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