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Dealing with Ebstein's anomaly

Published online by Cambridge University Press:  29 October 2013

Lianne M. Geerdink  and
Livia Kapusta
Lianne M. Geerdink*
Affiliation:
Department of Pediatrics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Livia Kapusta
Affiliation:
Pediatric Cardiology Unit, E. Wolfson Medical Centre, Holon, Israel Children's Heart Centre, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
*
Correspondence to: L. M. Geerdink, Department of Pediatrics, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands. Tel: 0031-6-47470386; Fax: 0031-24-3619348; E-mail: lgeerdink@gmail.com
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Abstract

Ebstein's anomaly is a complex congenital disorder of the tricuspid valve. Presentation in neonatal life and (early) childhood is common. Disease severity and clinical features vary widely and require a patient-tailored treatment. In this review, we describe the natural history of children and adolescents with Ebstein's anomaly, including symptoms and signs presenting at diagnosis. Current classification strategies of Ebstein's anomaly are discussed. We report on diagnostic methods for establishing the severity of disease that might enhance decision on the timing of surgical intervention. Furthermore, we describe different surgical options for severely ill neonates and multiple surgical interventions after infancy. Only with ample knowledge and understanding of the above, this complex and diverse group of patients can be correctly treated in order to improve not only duration, but also quality of life.

Keywords

Ebstein's anomalyclassificationdiagnosticsmanagementsurgeryoutcome
Type
Review Article
Information
Cardiology in the Young , Volume 24 , Issue 2 , April 2014 , pp. 191 - 200
Copyright
Copyright © Cambridge University Press 2013 

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