Surgical experience in adults with Ebstein's anomaly: Long-term results

The aim of this study was to review late results of the surgical treatment of Ebstein's anomaly with reconstruction and replacement in adults.

Medical records of 28 consecutive patients operated on between 1991 and 2014 were reviewed retrospectively. Surgical repair was performed in 19 (67.9%) patients (Hardy: two, Danielson: three, modified Danielson: six, Carpentier: three, Kay annuloplasty reinforced with ring: two), whereas tricuspid valve replacement was performed in nine patients (32.1%). Primary long-term outcomes consisted of right ventricular function, survival and freedom from re-operation. We evaluated the additional impacts of residual tricuspid insufficiency and type of surgery on survival.

In-hospital mortality rate was 7.1% (n = 2) due to low cardiac output status and sepsis. Patients showed a significant postoperative decrease in tricuspid regurgitation (p < 0.001), right atrial size (p < 0.001) and pulmonary hypertension (p = 0.002). The mean follow-up time was 140 ± 71.4 months, with a median of 126 months (105 - 192). Late mortality occurred in two patients and there was no significant difference in terms of survival based on residual tricuspid insufficiency (p = 0.57) and type of surgery (p = 0.094). Overall survival rates were 89.3, 85.4, 85.4 and 68.3% at five, 10, 15 and 20 years, respectively.

Although complex leaflet reconstruction techniques have evolved to achieve a more physiological and durable repair, both approaches can be performed safely on specific patients and can be alternated, with acceptable rates of survival and re-operation.