Management and outcome of Ebstein's anomaly in children.
Cardiol Young
; 23(1): 27-34, 2013 Feb.
Article
en En
| MEDLINE
| ID: mdl-22417890
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Anomalía de Ebstein
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
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Incidence_studies
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Observational_studies
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Risk_factors_studies
Límite:
Child
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Child, preschool
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Humans
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Infant
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Newborn
Idioma:
En
Revista:
Cardiol Young
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
PEDIATRIA
Año:
2013
Tipo del documento:
Article
País de afiliación:
Suiza