Cardiology in the Young

Table of Contents - February 2013 - Volume 23, Issue 01  

Original Article

Management and outcome of Ebstein's anomaly in children

Angela Oxeniusa1a2 c1, Christine H. Attenhofer Josta3, René Prêtrea2a4, Hitendu Davea2a4, Urs Bauersfelda1a2, Oliver Kretschmara1a2, Burkhardt Seiferta5, Christian Balmera1a2 and Emanuela R. Valsangiacomo Buechela1a2

a1 Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland

a2 Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland

a3 Cardiovascular Centre Zurich, Klinik im Park, Zurich, Switzerland

a4 Cardiothoracic Surgery, University Children's Hospital, Zurich, Switzerland

a5 Division of Biostatistics, Institute of Social and Preventive Medicine, University of Zurich, Zurich, Switzerland

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.

(Received June 28 2011)

(Accepted February 03 2012)

(Online publication March 15 2012)

Keywords

  • Congenital heart defect;
  • tricuspid valve;
  • intervention

Correspondence:

c1 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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