Outcome of Cardiac Surgery in Patients 50 Years of Age or Older With Ebstein Anomaly

Objectives This study sought to analyze the presentation, surgical procedures, and outcomes in patients ≥50 years of age with Ebstein anomaly (EA). Background Data on management and surgical outcomes in older patients with EA are limited. Methods Operative and clinical data from patients with EA ≥50 years of age undergoing cardiac surgery at our center between October 1980 and January 2010 were analyzed. Results During the study period, 89 procedures were performed in 81 patients with EA (63% women; mean [range] age 59 [50 to 79] years). Pre-operative symptoms included palpitations (n = 69), edema (n = 30), and previous stroke/transient ischemic attack (n = 21). Seventy-six patients (85%) had functional class III or IV symptoms, and 13 (16%) had previous cardiac surgery. Tricuspid valve surgery was necessary in 87 of the 89 procedures (98%): replacement in 65 (73%) and repair in 22 (25%). Three early deaths occurred (4%). On long-term follow-up (available in 73 of 78 early survivors), 63 patients (89%) had improved functional class and 13 patients died (19%). The 20-year survival was 65% versus 74% for age- and sex-matched controls (p = 0.001). The best predictors of late death were lack of post-operative improvement and older age at surgery. Conclusions Although cardiac surgery in patients with EA ≥50 years of age was often complex, early mortality was low (4%) when surgery was performed at an experienced center. Long-term survival was good, although less than expected. These data suggested that surgery in older patients with EA may have to be performed earlier.