Open Access

ARTICLE

Mid-Term Outcome of Surgical Treatment in Pediatric Patients with Ebstein’s Anomaly: A Single-Center Cohort Study

by Jiachen Li, Xianchao Jiang, Sim-eng Zhang, Jinyang Liu, Yajuan Zhang, Jun Yan, Shoujun Li, Qiang Wang^*

Pediatric Cardiac Center, Department of Cardiac Surgery, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100037, China

* Corresponding Author: Qiang Wang. Email:

Congenital Heart Disease 2020, 15(5), 387-397. https://doi.org/10.32604/CHD.2020.013127

Received 27 July 2020; Accepted 15 August 2020; Issue published 23 September 2020

Abstract

Background: Ebstein’s anomaly (EA) is a malformation of the tricuspid valve (TV) and myopathy of the right ventricle (RV). Surgery is now the main treatment for the defect. Objective: To summarize our surgical results and experience based on patients with EA who were under 7 years of age and treated with different surgical treatments. Design: Single-center cohort study of Ebstein’s anomaly. Setting: China. Participants: 80 patients under 7 years old who were diagnosed of EA and underwent different surgical treatments were consecutively enrolled in our research from January 2010 to December 2019. 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 mid-term survival of the total cohort, 1.5-ventricular repair and biventricular repair was 82.35%, 91.67% and 100%, respectively. The mid-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 mid-term valve regurgitations 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 mid-term follow-up (p = 0.036). Conclusion: The overall effect of surgical treatment for EA was good, and most patients could receive biventricular repair at an early stage. The reoperation rate and mid-term mortality were both low and TR was significantly improved.

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