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

ABSTRACT

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