Mid-term Outcome Of Surgical Treatment In Pediatric Patients With Ebstein's Anomaly: A Single-center Cohort Study.

Background: Ebstein’s anomaly is a malformation of the tricuspid valve and myopathy of the right ventricle. Surgery is now the main treatment for the defect. To summarize our surgical results and experience based on patients with Ebstein’s anomaly who were under 7 years of age and treated with different surgical treatments. Materials and Methods: From January 2010 to December 2019, 80 patients under 7 years old who were diagnosed of Ebstein’s anomaly and underwent different surgical treatments were consecutively enrolled and followed up in detail. Results: The median age of the 80 patients at the time of surgery was 3.63 years. Sixty-four (80.00%) patients underwent biventricular repair while 13 (16.25%) underwent 1.5-ventricle repair. With the median follow-up 27.50 months, the long-term survival of the total cohort, 1.5-ventricular repair and biventricular repair was 82.35%, 91.67% and 100%, respectively. The long-term freedom from reoperation rate was 97.50%, 92.31% and 98.44%, respectively. Mild, moderate and severe TR before surgery occurred in 6 (7.50%), 18 (22.50%) and 56 (70.00%), respectively. The early outcomes of 78 patients were 65 (83.33%), 11 (14.11%) and 2 (2.56%); the mid-term outcomes of 72 patients were 49 (68.06%), 19 (26.38%) and 4 (5.56%). Both early and long-term valve regurgitation were significantly decreased (p< 0.001) compared with preoperative condition. No more severe regurgitation occurred (p=0.404), though some early mild regurgitation became acceptable moderate regurgitation during long-term follow-up (p=0.036). Compared with Carpentier procedure, cone procedure had better long-term effect, while the effect of whole-valve technique needed more operation and long-term follow-up. Conclusion: The reoperation rate and mid-term mortality of surgical treatment for Ebstein’s anomaly were both low, tricuspid regurgitation was significantly improved during mid-term follow up. Cone procedure had the best mid-term effect among anatomic repair.