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Published on: February 8, 2022
Early and medium-term results for repair of Ebstein anomaly
Jonathan M Chen1, Ralph S Mosca, Karen Altmann
1Division of Pediatric Cardiac Surgery, Children's Hospital of New York, Columbia University College of Physicians and Surgeons, NY 10032, USA. jmc23@columbia.edu
Insights
Primary repair of Ebstein anomaly shows good functional outcomes in children, with adults often needing tricuspid valve replacement. Radiofrequency ablation is effective for arrhythmias during Ebstein anomaly repair.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Ebstein anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Surgical repair aims to correct valve function and improve patient outcomes.
- Outcomes can vary significantly between pediatric and adult populations.
Purpose of the Study:
- To evaluate the early and medium-term results of primary Ebstein anomaly repair.
- To compare outcomes between pediatric and adult patients.
- To assess the role of adjunctive procedures like radiofrequency ablation.
Main Methods:
- Retrospective review of 25 patients (19 children, 6 adults) undergoing Ebstein anomaly repair from 1990-2002.
- Analysis of functional, demographic, and echocardiographic parameters pre- and post-operatively.
- Repair technique included ventricular plication and valve leaflet reimplantation.
Main Results:
- 18 children (95%) and 5 adults (83%) showed improved exercise capacity post-repair.
- 3 patients required reoperation for right ventricular overload or tricuspid regurgitation.
- 2 children experienced sudden death; 2 adults required tricuspid valve replacement years after initial repair.
Conclusions:
- Ebstein anomaly repair yields good functional results in children, attributed to reduced ventricular volume loading and plasticity.
- Adults experienced less durable valve repairs, often necessitating subsequent tricuspid valve replacement.
- Intraoperative radiofrequency ablation is valuable for managing arrhythmias during Ebstein anomaly repair.
Objectives:
We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children.
Methods:
The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation.
Results:
A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair.
Discussion:
Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.
