RESUMO
OBJECTIVE: To evaluate efficacy of one-step and two-step surgery approach in patients with arrhythmias combined with Ebstein's anomaly. METHODS: Fifty-three patients with Ebstein's anomaly combined with tachyarrhythmias (58.5% men, 41.5% women, mean age 21.6+/-10.7 years) were operated on. In group A (32 patients), one-step surgical correction (simultaneous intraoperative elimination of arrhythmias and congenital heart defect repair) was performed, whereas in group B (21 patients), two-step surgery was performed with initial elimination of arrhythmogenic substrate by transcatheter radiofrequency ablation (first step) and following surgical repair of congenital heart defect (second step). RESULTS: In group A, total hospital mortality was 3.1% (1 patient) due to initial severe condition of this patient. One-step surgery was effective in 93.5% of cases. Mortality was not observed in group B. Efficacy of transcatheter radiofrequency ablation was 76.2%. CONCLUSIONS: One-stage and two-stage surgery of arrhythmias and Ebstein's anomaly are highly effective. First step of surgery of combined pathology reduces cardiopulmonary bypass time, complications and mortality while performing the second step of congenital heart defect surgery. However, simultaneous approach (one-step surgery) is better in terms of arrhythmia elimination.