The surgical treatment strategy and medium and long-term follow-up of Ebstein’s anomaly
10.3760/cma.j.cn112434-20191121-00417
- VernacularTitle:三尖瓣下移畸形的外科治疗策略和中远期随访
- Author:
Lun LI
1
;
Xueyong YANG
;
Xiaoyong JING
;
Junwu SU
;
Pei CHENG
;
Yinglong LIU
;
Xiangming FAN
Author Information
1. 首都医科大学附属北京安贞医院小儿心脏中心 100029
- From:
Chinese Journal of Thoracic and Cardiovascular Surgery
2020;36(6):321-325
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the surgical treatment strategy and the results of medium and long-term follow-up of Ebstein's anomaly(EA) through analyzing 127 patients.Methods:From January 2010 to Decembe 2018, 127 patients with EA underwent operation. There were 59 male and 68 female patients, with age of(9.5±3.7) years(0.67-18.0 years), weight of(27.1±11.7) kg(6.4-76.0 kg).13 cases were diagnosed as WPW syndrome by Echocardiography. There were 11 cases of VSD, 42 cases of ASD, 34 cases of PFO, 6 cases of PS and 127 cases of TI. The surgical strategy included 92 cases of biventricular repair(including 20 cases retained atrial shunt) and 35 cases of one and a half ventricular repair(including 8 cases retained atrial shunt). Except 2 patients underwent simple tricuspid valve repair(including 1 case of tricuspid annuloplasty ring), 125 EA patients in this group were all treated with cone reconstruction method for atrioventricular folding and tricuspid valvuloplasty.Results:6 patients died within perioperative period because of low cardiac output, pulmonary infection and cerebral hemorrhage, with a mortality of 4.7%. The complete atrioventricular block occurred in 2 patients and pacemaker were implanted postoperatively. 7 patients(5.5%) underwent pericardial fenestration due to pericardial effusion. All the other patients recovered successfully and were discharged from hospital without serious complications. The echocardiographic result indicated that the degree of tricuspid insufficiency were significantly reduced and cardiac function were improved before all patients discharged. The ratio of functional right ventricle to atrialized right ventricle(FRV/ARV) in the whole group was 0.87±0.37. FRV/ARV ratios of biventricular repair group and one and a half ventricular repair group were 1.12±0.39 and 0.48±0.28, respectively. And the difference between the two groups was statistically significant( t=5.685, P< 0.001). 110 cases were followed up in 3 to 96 months. 3 patients(2.7%) died during medium and long-term follow-up. Conclusion:The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. Surgical treatment strategies should be decided according to the condition of tricuspid valve, ratio of FRV/ARV. Surgical treatment has a good effect for Ebstein’s anomaly in the medium and long-term.
