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The outcomes of operations for 539 patients with Ebstein anomaly
Morgan L Brown1, Joseph A Dearani, Gordon K Danielson
1Division of Cardiovascular Surgery, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minn 55905, USA.
Insights
This study reviewed long-term outcomes for Ebstein anomaly patients undergoing cardiac surgery. Surgical treatment offers low perioperative mortality and good long-term survival for Ebstein anomaly (EA).
Area of Science:
- Cardiology
- Cardiac Surgery
- Congenital Heart Disease
Background:
- Ebstein anomaly (EA) is a rare congenital heart defect affecting the tricuspid valve.
- Surgical intervention is often necessary for symptomatic EA patients.
- Understanding long-term outcomes is crucial for patient management.
Purpose of the Study:
- To evaluate the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery.
- To identify factors associated with mortality and survival after surgery for EA.
Main Methods:
- Retrospective review of patient records for Ebstein anomaly patients undergoing cardiac surgery.
- Exclusion of patients with specific complex congenital heart conditions.
- Data collection on perioperative and long-term outcomes, including survival and patient-reported health status.
Main Results:
- 539 patients underwent 604 cardiac operations between 1972 and 2006.
- 30-day mortality was 5.9% (2.7% after 2001); 10-year and 20-year survival rates were 84.7% and 71.2%, respectively.
- Multivariate analysis identified predictors of mortality and survival, including hematocrit, valve repair/replacement, and preoperative rhythm.
Conclusions:
- Cardiac surgery for Ebstein anomaly can be performed with low perioperative mortality.
- Both tricuspid valve repair and replacement are associated with favorable long-term survival.
- Factors such as ventricular dysfunction, elevated hematocrit, and outflow tract obstruction are associated with poorer outcomes.
Objective:
Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution.
Methods:
Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded.
Results:
From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations. The mean age at the time of the initial operation at our institution was 24 years (range, 8 days-79 years). Three hundred seventeen of the patients were female. One hundred forty-three (26.5%) patients had a prior invasive cardiac procedure before coming to Mayo Clinic. At the time of the first operation at Mayo Clinic, 182 patients had tricuspid valve repair, and 337 had tricuspid valve replacement. The 30-day mortality was 5.9% for the entire cohort (2.7% after 2001). Late survival was 84.7% at 10 years and 71.2% at 20 years. In a multivariate analysis of overall mortality for the patients' first operation at Mayo Clinic, increased hematocrit values, pulmonary valve stenosis, tricuspid valve replacement, absence of ablation of an accessory pathway, miscellaneous arrhythmia procedure, branch pulmonary artery enlargement, need for mechanical support postoperatively, emergency chest opening in the intensive care unit, and absence of sinus rhythm at dismissal were all predictive of mortality. When only preoperative characteristics were included, increased hematocrit values, mitral valve regurgitation requiring surgical intervention, prior cardiac procedure, and moderate-to-severe to severe reduction in right ventricular systolic function were associated with mortality. Preoperative sinus rhythm and an accessory pathway were associated with survival. Patients rated their health as excellent or good (New York Heart Association class I or II) in 83% of surveys returned.
Conclusion:
Ebstein anomaly can be surgically treated with low perioperative mortality. Both tricuspid valve repair and tricuspid valve replacement are associated with good long-term survival. Risk factors for poorer outcome included right, and/or left ventricular systolic dysfunction; increased hemoglobin/hematocrit values; male sex; right ventricular outflow tract obstruction; or hypoplastic pulmonary arteries.