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.
- Ebstein anomaly
- tricuspid valve surgery
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine