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Updated: Jun 20, 2026
Vessel-sparing Excision and Primary Anastomosis
Published on: January 7, 2019
Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome
Catalin Constantin Badiu1, Christian Schreiber, Jürgen Hörer
1German Heart Centre Munich, Department of Cardiovascular Surgery, Clinic at the Technical University Munich, Munich, Germany. badiu@dhm.mhn.de
Insights
For Ebstein's anomaly, tricuspid valve repair is often successful, offering good long-term survival. Timely surgery is crucial before significant heart enlargement or functional decline occurs.
Area of Science:
- Cardiology
- Cardiac Surgery
- Congenital Heart Disease
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Surgical interventions for Ebstein's anomaly have evolved, but optimal timing remains unclear.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical repair versus replacement for Ebstein's anomaly.
- To identify factors influencing mortality and re-operation rates.
Main Methods:
- Retrospective analysis of 122 patients with Ebstein's anomaly who underwent surgery between 1976 and 2007.
- Tricuspid valve repair was performed in 90.2% of patients; replacement in 9.8%.
- Mean follow-up was 10.5 years.
Main Results:
- Hospital mortality was low (1.5%).
- Long-term survival was comparable between repair and replacement groups.
- Significant risk factors for mortality included NYHA class >II and cardiothoracic ratio >0.6.
- Age <=12 years and cardiothoracic ratio >0.6 were risk factors for re-operation.
Conclusions:
- Tricuspid valve repair is feasible and safe for most Ebstein's anomaly patients.
- Optimal surgical timing is essential, ideally before significant cardiomegaly or functional deterioration.
- Early intervention improves long-term survival and reduces re-operation rates.
Objective:
Various surgical valve repair and replacement techniques have been developed over the past decades for patients with Ebstein's anomaly. Determination of the appropriate moment for surgery, however, has not been elucidated clearly enough.
Methods:
From 1976 to 2007, 130 patients (mean age 23.8+/-17.8 years, range: 1 month to 73.6 years) underwent surgery for Ebstein's anomaly at our centre. Four patients (3.0%), who underwent univentricular palliation, and four (3.0%), who only had an atrial septal defect closure, were excluded. In 110/122 (90.2%) patients, a primary tricuspid valve repair was feasible. Valve replacement was necessary in 12 (9.8%). Mean follow-up time was 10.5+/-9.1 years (94.3% complete, 1284 patient years).
Results:
There were two (1.5%) hospital deaths. Overall survival was 87.2%+/-3.6%, 85.1%+/-4.1% and 81.2%+/-5.4% at 10, 20 and 25 years, respectively, without significant difference between the repair and replacement group (p=0.31). The New York Heart Association functional class >II (p=0.01) and cardiothoracic ratio >0.6 (p=0.02) were significant risk factors for mortality. Overall freedom from re-operation was 79.9+/-4.6%, 61.9+/-6.8% and 58.0+/-7.4% at 10, 20 and 25 years, respectively. Age
Conclusions:
Repair, as opposed to replacement, is feasible in the vast majority of patients presenting with Ebstein's anomaly with a low early mortality rate. Outcome, in terms of survival and freedom from re-operation in the long term is determined by the clinical state at the time of surgery. Therefore, timely operation is warranted before significant cardiomegaly develops and functional status deteriorates.
