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Management and outcome of Ebstein's anomaly in children

Published online by Cambridge University Press:  15 March 2012

Angela Oxenius ,
Christine H. Attenhofer Jost ,
René Prêtre ,
Hitendu Dave ,
Urs Bauersfeld ,
Oliver Kretschmar ,
Burkhardt Seifert ,
Christian Balmer  and
Emanuela R. Valsangiacomo Buechel
Angela Oxenius*
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Christine H. Attenhofer Jost
Affiliation:
Cardiovascular Centre Zurich, Klinik im Park, Zurich, Switzerland
René Prêtre
Affiliation:
Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland Cardiothoracic Surgery, University Children's Hospital, Zurich, Switzerland
Hitendu Dave
Affiliation:
Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland Cardiothoracic Surgery, University Children's Hospital, Zurich, Switzerland
Urs Bauersfeld
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Oliver Kretschmar
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Burkhardt Seifert
Affiliation:
Division of Biostatistics, Institute of Social and Preventive Medicine, University of Zurich, Zurich, Switzerland
Christian Balmer
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Emanuela R. Valsangiacomo Buechel
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
*
Correspondence to: Dr A. Oxenius, MD, Department of Pediatric Cardiology, University Children's Hospital, Steinwiesstrasse 75, 8032 Zurich, Switzerland. Tel: +41 44 266 70 22; Fax: +41 44 266 79 81; E-mail: angela.oxenius@kispi.uzh.ch
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Abstract

Objectives

To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.

Background

Data on long-term outcome of children with Ebstein's anomaly are scarce.

Methods

Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.

Results

A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1–16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.

Conclusion

In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Keywords

Congenital heart defecttricuspid valveintervention
Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 1 , February 2013 , pp. 27 - 34
Copyright
Copyright © Cambridge University Press 2012

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