Ebstein anomaly of the tricuspid valve in an adult cohort

grantor: University of Toronto'Objective.' We examined the spectrum of adults with Ebstein anomaly of the tricuspid valve. 'Methods.' Clinical data, EKG, chest x-rays, echocardiograms and surgical details were reviewed in patients with Ebstein anomaly at the University of Toronto Congenital Cardiac Center for Adults. 'Results.' Seventy-four patients (27 M, 47 F) were seen at our clinic at a mean age of 33.2 ± 13.6 years and were followed for a mean of 7.5 ± 5.8 years. Seven patients died. Thirty-eight patients (51%) presented with or developed sustained arrhythmia (atrial flutter/fibrillation n = 27, supraventricular tachycardia due to WPW n = 16); 7 of these patients with arrhythmia experienced transient ischemic attacks. Tricuspid valve surgery was undertaken in 24 patients; NYHA class improved in all 23 survivors. Of the 12 patients that experienced atrial flutter/fibrillation before surgery, arrhythmia persisted in 6. Only 2 of these 6 patients had intraoperative arrhythmia ablation. The other 6 patients undergoing ablative procedures converted to sinus rhythm. Right atrial size (p < 0.02) and the presence of atrial septal defect (p < 0.02) are independent predictors of atrial flutter/fibrillation (using Cox regression analysis), whereas cardiothoracic ratio (p < 0.03) is predictive of death. 'Conclusions.' Atrial flutter/fibrillation is common in adults with Ebstein anomaly, and relates to RA size. Surgery for Ebstein anomaly results in symptomatic improvement. Atrial flutter/fibrillation may persist without concomitant intraoperative arrhythmia ablation.M.Sc