Management and outcome of Ebstein's anomaly in children.

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.

journal_name

Cardiol Young

journal_title

Cardiology in the young

authors

Oxenius A,Attenhofer Jost CH,Prêtre R,Dave H,Bauersfeld U,Kretschmar O,Seifert B,Balmer C,Valsangiacomo Buechel ER

doi

10.1017/S1047951112000224

subject

Has Abstract

pub_date

2013-02-01 00:00:00

pages

27-34

issue

1

eissn

1047-9511

issn

1467-1107

pii

S1047951112000224

journal_volume

23

pub_type

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