Reconstructive surgery for Ebstein anomaly: three decades of experience

OBJECTIVES: Since 1988, our centre employs vertical plication repair with deattachment and reattachment of the tricuspid valve for Ebstein anomaly. This study describes the characteristics and long-term outcomes of our single-centre cohort.

METHODS: Data from all patients operated on between 1988 and 2016 were retrospectively collected. Kaplan-Meier analyses were done for survival data and mixed models were used to analyse longitudinally collected clinical and echocardiography data.

RESULTS: Thirty-six patients (mean age: 25.4 ± 15.9 years, 36% male) were operated on using the Carpentier-Chauvaud 21 (58%) or Cone repair 15 (42%). One patient (3%) died in hospital. Two late deaths were observed, yielding a survival of 97 ± 3% at 25 years. Reoperation was performed in 6 patients after a mean follow-up of 14.1 ± 10.3 years, resulting in a freedom of reoperation of 80 ± 8% at 25 years. During follow-up, predicted probability of being in New York Heart Association III/IV did not exceed 10%. Modelling longitudinal evolution of tricuspid regurgitation showed no major changes over time. Additionally, a rigid ring repair was associated with a higher probability of tricuspid regurgitation, especially after the first years after the operation. A full Cone repair was associated with less progression of tricuspid regurgitation over time.

CONCLUSIONS: Repair of Ebstein abnomaly is associated with low mortality and morbidity, acceptable reoperation rate and excellent valve function over time, especially in patients with completed Cone repair. Therefore, we conclude that in our centre, repair of Ebstein abnomaly is a durable technique to treat patients.