Valve-preserving treatment of Ebstein's anomaly: perioperative and follow-up results

N Kupilik1, P Simon, R Moidl

  • 1Department of Cardiothoracic Surgery, University of Vienna, Austria.

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.
Abstract

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