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Management and outcome of Ebstein's anomaly in children
Angela Oxenius1, Christine H Attenhofer Jost, René Prêtre
1Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland. angela.oxenius@kispi.uzh.ch
Insights
Ebstein's anomaly in children often requires intervention, but outcomes are generally good with low peri-operative mortality and high long-term survival rates. Early diagnosis is key, though newborns needing intervention may face worse prognoses.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Long-term outcome data for pediatric Ebstein's anomaly are limited.
- Ebstein's anomaly is a rare congenital heart condition affecting the tricuspid valve.
Purpose of the Study:
- To evaluate the clinical presentation, treatment strategies, and outcomes of children diagnosed with Ebstein's anomaly.
- To provide insights into the long-term prognosis for pediatric patients with this condition.
Main Methods:
- Retrospective analysis of 42 children with Ebstein's anomaly treated between 1979 and 2009.
- Primary outcomes assessed were patient survival and the need for cardiac or catheter interventions.
Main Results:
- Ebstein's anomaly was diagnosed in infants and children, with 90% having associated cardiac anomalies.
- 33 patients (79%) required interventions, including cardiac surgery (50%) and catheter-guided procedures.
- Overall mortality was 14%, with a 10-year survival rate of 85.3%. Peri-operative mortality for surgeries was 4%.
Conclusions:
- Ebstein's anomaly in children is typically diagnosed within the first year of life.
- While interventions are common, peri-operative mortality is low, and long-term survival is favorable.
- Symptomatic newborns requiring intervention may experience poorer outcomes.
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.
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