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Updated: Nov 4, 2025
Vessel-sparing Excision and Primary Anastomosis
Published on: January 7, 2019
Surgical treatment of Ebstein anomaly in pediatric patients: A 10-year single-center study
Shiraslan Bakhshaliyev1, Zeynep Gülben Kük Özalp1, Mustafa Güneş1
1Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Education and Research Hospital, Istanbul, Turkey.
Insights
Surgical strategies for Ebstein anomaly (EA) in pediatric patients yielded good early and long-term outcomes. Biventricular repair was common, with low mortality and re-intervention rates, demonstrating effective treatment for EA.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Repair
Background:
- Ebstein anomaly (EA) is a complex congenital heart defect requiring surgical intervention.
- Treatment strategies for pediatric EA vary, necessitating evaluation of outcomes.
Purpose of the Study:
- To report early and long-term results of surgical interventions for pediatric Ebstein anomaly.
- To analyze the effectiveness of different surgical methods employed at a single center.
Main Methods:
- Retrospective analysis of 29 consecutive pediatric patients undergoing EA surgery (Feb 2011-Feb 2020).
- Evaluation of surgical approaches including univentricular, 1.5 ventricular, and biventricular repairs.
- Assessment of tricuspid valve (TV) repair techniques: Cone reconstruction (CR), non-Cone repair, and TV replacement.
Main Results:
- A total of 40 operations were performed across 29 patients.
- Biventricular repair was the most common approach (65.5%).
- Early mortality was 3.4% (1 patient); no long-term mortality observed. Low rates of moderate/advanced tricuspid regurgitation post-discharge and acceptable re-intervention rates were noted.
Conclusions:
- Surgical treatment of pediatric EA, while challenging, demonstrates favorable overall results.
- Biventricular repair is frequently utilized, even in neonates/infants.
- Acceptable survival rates, low early mortality, and minimal long-term morbidity/re-intervention highlight treatment success.
Objective:
We report the early and long-term results of the strategies and surgical methods used in our center to treat pediatric patients who underwent surgical intervention to correct Ebstein anomaly (EA) in our center.
Materials And Methods:
In our study, a consecutive sample of 29 patients who underwent surgery for EA between February 2011 and February 2020 in our center were evaluated retrospectively.
Results:
The 29 patients underwent a total of 40 operations. Univentricular repair was performed in 5 (17.2%), 1.5 ventricular repair in 5 (17.2%), and biventricular repair in the remaining 19 (65.5%) patients. Cone reconstruction (CR) was performed in eight (27.5%), non-Cone tricuspid valve (TV) repair technique in five (17.2%), ring annuloplasty in two (6.9%), and TV replacement in two patients (6.9%) who had undergone biventricular repair. In two patients (6.9%), only close an atrial septal defect. Two (6.9%) patients underwent a second operation for advanced tricuspid regurgitation (TR) in the early period. None of the 15 patients who underwent CR and TV plasty had moderate or advanced TR before discharge. Early mortality was seen in 1 (3.4%) patient. The mean follow-up period of the patients was 48.4±27.6 months. Three (10.7%) of the patients who were discharged after their first operation later underwent a second operation for TV regurgitation in the long term. No mortality was observed in any patient during long-term follow-up.
Conclusion:
Surgical treatment of EA is difficult, but its overall results are good. The anatomical repair rate is lower in neonatal and infant patients requiring surgery, but most of these patients underwent biventricular repair. Our long-term results demonstrated an acceptable survival rate, low mortality in the early postoperative period, and low incidence of re-intervention and morbidity.
