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Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience
Umar S Boston1, Joseph A Dearani, Patrick W O'Leary
1Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA.
Insights
Tricuspid valve repair for Ebstein's anomaly in young children shows low mortality and good long-term durability. Achieving mild tricuspid regurgitation post-surgery is key to minimizing reoperations and ensuring an excellent quality of life.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Surgical repair in young children presents unique challenges for early and late outcomes.
Purpose of the Study:
- To evaluate the early and late results of tricuspid valve repair and annuloplasty in pediatric patients with Ebstein's anomaly.
- To identify risk factors for mortality and reoperation.
Main Methods:
- A retrospective analysis of 52 children undergoing tricuspid valve repair and annuloplasty for Ebstein's anomaly between 1974 and 2003.
- Mean follow-up of 12.2 years, assessing survival, reoperation rates, and functional status.
Main Results:
- Early mortality was 5.8%, with no deaths since 1984. Younger age and lower weight were risk factors.
- Actuarial survival at 15 years was 89.9%. Freedom from reoperation at 15 years was 61.4%.
- Moderate or greater tricuspid regurgitation post-repair was a risk factor for reoperation. 89% of patients were in NYHA class I or II at late follow-up.
Conclusions:
- Tricuspid valve repair for Ebstein's anomaly in children offers low mortality and durable results.
- Minimizing tricuspid regurgitation during surgery is crucial for long-term success and avoiding reoperation.
- Most patients achieve an excellent quality of life with successful repair, without developing tricuspid stenosis.
Background:
The purpose of this study was to examine early and late outcome of tricuspid valve repair for Ebstein's anomaly in young children.
Methods:
Between October 1974 and November 2003, 52 children (25 boys) underwent tricuspid valve repair and annuloplasty for Ebstein's anomaly. Mean age was 7.1 +/- 3.9 years (range, 5 months to 12 years). Concomitant procedures included atrial septal defect closure (n = 46), division of accessory conduction pathways (n = 4), ventricular septal defect closure (n = 3), and other (n = 7).
Results:
Early mortality was 5.8% (3 of 52 patients; no mortality since 1984, n = 31). Risk factors were age younger than 2.5 years (p = 0.03) and weight less than 10.7 kg (p = 0.03). Morbidity included transient atrial (n = 11) and ventricular arrhythmias (n = 5), and early reoperation in 3 patients. There was no need for a permanent pacemaker. Mean follow-up was 12.2 +/- 7.4 years (maximum, 24.3 years). Actuarial survival at 5, 10, and 15 years was 92.3% +/- 3.7%, 89.9% +/- 4.3%, and 89.9% +/- 4.3%, respectively. Freedom from all reoperations at 5, 10, and 15 years was 91.0% +/- 4.3%, 76.9% +/- 6.8%, and 61.4% +/- 8.8%, respectively. Moderate (grade II) or more tricuspid regurgitation on dismissal echocardiogram was the only risk factor for reoperation (p = 0.04). Tricuspid stenosis did not occur in any patient. At late follow-up, 89% of patients were in New York Heart Association class I or II.
Conclusions:
Ebstein's anomaly in young children can now be repaired with low mortality and good tricuspid valve durability. Tricuspid regurgitation at the completion of operation should be mild or less to minimize need for reoperation. Tricuspid valve repair and annuloplasty did not result in stenosis despite somatic growth. Most patients enjoy an excellent quality of life.
