[Operation of Ebstein anomaly in adulthood - our experience]

The outcome of the operation of the Ebstein anomaly in adulthood depends on the experience of the surgical team.

We operated 38 adult patients with Ebstein anomaly at the age of 34.8 ± 12.7 (19-63) years at the Department of Cardiac Surgery, Hospital Na Homolce, Prague, in the period of 2005-2013. The majority of patients (71 %) had Ebstein anomaly type C or D. The 30-days postoperative mortality was 5.2 %. In the long-term follow-up 2 other patients died. Tricuspid valve repair was performed in 45 %, bioprosthesis was implanted in 53 %. Concomitant bi-directional cavo-pulmonary anastomosis was performed in 31.5 %, mitral valve repair in 8 %, closure of atrial communication in 83 %, right-sided MAZE or istmus cryo-ablation in 39 %. After the operation we found improvement of the functional NYHA class (from 2.2 ± 0.7 to 1.7 ± 0.6; p < 0.0001) as well as decrease of tricuspid regurgitation (from grade 3.8 ± 0.4 to 0.9 ± 1; p < 0.0001). The ejection fraction of the right ventricle (RVEF) improved in 86 % of patients, in 14 % RVEF decreased or did not change (RVEF before operation 36 ± 10.5 %, after operation 42 ± 9.5 %, p = 0.001). In the long-term follow-up we found dysfunction of the tricuspid valve repair in 12 % and degeneration of the bioprosthesis in 15 %. Among 86 adult patients with Ebstein anomaly from our database the mortality was significantly higher in unoperated compared to operated patients (26 % vs 12 %, p = 0.006). Patients with cyanosis had high mortality regardless of surgery (40 % with and 83 % without operation). Reoperation after surgery in childhood or adulthood was necessary in 20 % of adults.

The adult patients with Ebstein anomaly should be examined in a specialized center even if the symptoms are mild. The operation should be performed by a team with good results and experience in surgery and post-operative care in Ebstein anomaly. According to our knowledge Hospital Na Homolce represents such specialized center for Czech Republic.