title: Management and outcome of Ebstein's anomaly in children.
abstract: OBJECTIVE
  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%.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland. angela.oxenius@kispi.uzh.ch
authors.forename: Angela
authors.initials: A
authors.lastname: Oxenius
authors.forename: Christine H
authors.initials: CH
authors.lastname: Attenhofer Jost
authors.forename: René
authors.initials: R
authors.lastname: Prêtre
authors.forename: Hitendu
authors.initials: H
authors.lastname: Dave
authors.forename: Urs
authors.initials: U
authors.lastname: Bauersfeld
authors.forename: Oliver
authors.initials: O
authors.lastname: Kretschmar
authors.forename: Burkhardt
authors.initials: B
authors.lastname: Seifert
authors.forename: Christian
authors.initials: C
authors.lastname: Balmer
authors.forename: Emanuela R
authors.initials: ER
authors.lastname: Valsangiacomo Buechel
mesh_terms: D006328:Cardiac Catheterization / Q000706:statistics & numerical data; D006348:Cardiac Surgical Procedures / Q000706:statistics & numerical data; D017115:Catheter Ablation / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004437:Ebstein Anomaly / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D013610:Tachycardia / Q000150:complications / Q000628:therapy; D016896:Treatment Outcome