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Valve-preserving treatment of Ebstein's anomaly: perioperative and follow-up results
Insights
Surgical repair of Ebstein's anomaly improves tricuspid insufficiency and right-ventricular function. Early correction, especially in children, enhances quality of life and avoids severe heart failure.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve (TV), often causing severe tricuspid insufficiency (TI).
- Patients frequently present with additional congenital cardiac defects such as atrial septal defect (ASD) and ventricular septal defect (VSD).
Purpose of the Study:
- To evaluate the efficacy of surgical repair for Ebstein's anomaly using modified Carpentier techniques.
- To assess the impact of surgical correction on tricuspid insufficiency, right-ventricular function, and overall quality of life.
Main Methods:
- Echocardiography was performed pre- and post-operatively, with follow-up assessments at mean intervals of 17 and 53 months.
- Ten patients (6 adults, 4 children) underwent tricuspid valve repair between 1989 and 1995.
- The surgical technique involved leaflet mobilization and ring implantation to improve valve coaptation.
Main Results:
- Preoperative severe tricuspid insufficiency (mean grade 3.2) was significantly reduced postoperatively (mean grade 2.0).
- Improvements were observed in right-ventricular function (mean score 2.8) and the ratio of atrialized chamber to functional right ventricle in 60% of patients.
- Most patients showed improved right-ventricular function and reduced tricuspid insufficiency at follow-up, with all experiencing enhanced quality of life.
Conclusions:
- Surgical correction of Ebstein's anomaly yields positive outcomes, including improved cardiac function and quality of life.
- Early surgical intervention, particularly in pediatric patients, is recommended to prevent the development of severe right heart failure.
Background:
Ebstein's anomaly is a rare congenital cardiac defect of the tricuspid valve (TV) leading to severe tricuspid insufficiency (TI).
Methods:
In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), operated between 1989 and 1995 echocardiography was performed pre and post repair and at follow-up. Patients were assessed in our institution at two cut-off points, resulting in a mean first follow-up of 17 +/-15 months and a mean second follow-up of 53+/-23 months. All patients had additional congenital cardiac defects (ASD,VSD). In all patients the TV was repaired by techniques described by Carpentier et al. with some modifications. The goal of this reparative attempt is to mobilize restricted leaflet tissue and aid coaptation through implantation of a ring.
Results:
Echocardiographically we were able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the size and function of the right ventricle and the atrialized chamber, the most advanced cases exhibiting a dilated right ventricle with poor contractility. There was severe preoperative TI ( mean grade 3.2 +/- 0.3). Postoperatively TI was significantly reduced to a mean grade of 2+/-0.2. 60% of the patients demonstrated an improvement in the ratio of atrialized chamber to functional right ventricle. Right-ventricular function was improved, the mean score being 2.8+/-0.1. At follow-up I and II right-ventricular function and tricuspid insufficiency was improved in most patients and all patients benefited in quality of life.
Conclusion:
These results suggest that surgical correction should not be delayed until severe right heart failure develops as, particularly in children, good results are achieved, improving the quality of life.