Chinese expert consensus on the surgical treatment of Ebstein anomaly in children and adults
- VernacularTitle:儿童和成人Ebstein畸形外科治疗中国专家共识
- Author:
Hao ZHANG
1
;
Keming YANG
2
;
Jimei CHEN
3
;
Liangwan CHEN
4
;
Shoujun LI
1
Author Information
1. Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, 200000, P. R. China
2. Fuwai Hospital, Chinese Academy of Medical Sciences, Beijing, 100037, P. R. China
3. Guangdong Provincial People's Hospital, Guangzhou, 510180, P. R. China
4. Union Hospital, Fujian Medical University, Fuzhou, 350001, P. R. China
- Collective Name:Congenital Heart Disease Committee of the National Expert Committee on Cardiovascular Diseases
- Publication Type:Journal Article
- Keywords:
Congenital heart disease;
Ebstein anomaly;
surgery;
treatment;
expert consensus
- From:
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
2025;32(12):1677-1685
- CountryChina
- Language:Chinese
-
Abstract:
Ebstein anomaly (EA) is a rare type of congenital heart defect. Its incidence ranges from 0.005‰ to 0.025‰ among live-born fetuses. It is characterized by the displacement of the septal and posterior leaflets of the tricuspid valve toward the apex of the right ventricle, along with the atrialization and thinning of the right ventricle. Based on the severity of these anatomical features, EA can be classified into four types. The degree of hemodynamic abnormalities mainly depends on factors such as the volume of the atrialized right ventricle, tricuspid regurgitation, and right ventricular function. The main clinical manifestations include: heart failure, cyanosis, and arrhythmia. Echocardiography is the first-choice examination method for confirming the diagnosis. In addition, cardiac magnetic resonance is recognized as the gold standard for evaluating tricuspid regurgitation index and right ventricular function, and it holds significant value in the preoperative diagnosis of EA, treatment decision-making, and postoperative follow-up. Surgical intervention is the primary treatment approach. Although multiple surgical methods exist, the current Cone reconstruction technique is the preferred surgical procedure for this disease. Based on evidence-based data from literature and expert opinions, this article provides a comprehensive summary and recommendations regarding the clinical classification, diagnostic criteria, surgical treatment strategies, management of complications, and prognosis evaluation of EA.
