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Management and outcome of Ebstein's anomaly in children

Oxenius, Angela ; Attenhofer Jost, Christine H. ; Prêtre, René ; Dave, Hitendu ; Bauersfeld, Urs ; Kretschmar, Oliver ; Seifert, Burkhardt ; Balmer, Christian ; Valsangiacomo Buechel, Emanuela R.

In: Cardiology in the Young, 2013, vol. 23, no. 1, p. 27-34

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    Titel
    • Management and outcome of Ebstein's anomaly in children
    Autor
    • Oxenius, Angela. Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland
    • Attenhofer Jost, Christine H.. Cardiovascular Centre Zurich, Klinik im Park, Zurich, Switzerland
    • Prêtre, René. Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
    • Dave, Hitendu. Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
    • Bauersfeld, Urs. Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland
    • Kretschmar, Oliver. Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland
    • Seifert, Burkhardt. Division of Biostatistics, Institute of Social and Preventive Medicine, University of Zurich, Zurich, Switzerland
    • Balmer, Christian. Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland
    • Valsangiacomo Buechel, Emanuela R.. Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland
    Dokumententyp
    • Postprint
    Sprache
    • Englisch
    Veröffentlicht in
    • Cardiology in the Young, 2013, vol. 23, no. 1, p. 27-34. Cambridge University Press
    Andere elektronische Ausgabe
    OAI-PMH-ID
    • oai:doc.rero.ch:301395
    Summary
    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