Vnitr Lek 2014, 60(4):335-340

Operation of Ebstein anomaly in adulthood - our experience

Jana Popelová^1,2,*, Roman Gebauer^1,2, Petr Pavel¹, Štěpán Černý¹, Pavel Jehlička¹, Petr Plášil¹, Ivo Skalský¹

¹ Centrum pro diagnostiku a léčbu vrozených srdečních vad u dospělých, vedoucí lékařka doc. MUDr. Jana Popelová, CSc., Kardiochirurgického oddělení Nemocnice Na Homolce Praha, přednosta prim. MUDr. Ivo Skalský, MBA

² Dětské kardiocentrum FN Motol Praha, přednosta a prim. prof. MUDr. Jan Janoušek, Ph.D.

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

Methods and results:
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.

Conclusion:
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.

Keywords: bidirectional cavo-pulmonary anastomosis; cyanosis; Ebstein anomaly of the tricuspid valve; tricuspid valve repair; operation of adults with congenital heart disease

Received: February 2, 2014; Published: April 1, 2014  Show citation