目的 分析成人三尖瓣下移畸形（EA）患者临床及心脏超声的特点，为临床提供更多有价值
的信息。方法 回顾性分析首都医科大学附属北京安贞医院于2002年1月至2018年8月收治的168例EA患者临
床及超声资料。结果 入组EA患者共168例，伴发心内或心外疾病患者总数127例（75.60%）。入组患者大
多接受三尖瓣畸形手术治疗及相关伴发疾病诊治，其中三尖瓣下移矫治术最多，共78例（46.43%），心功
能Ⅳ级患者均未进行手术。因心律失常或伴发心律失常入院共36例。住院期间总死亡人数3例（1.79%），
均为心功能Ⅲ级患者。EA患者中单纯三尖瓣隔叶发生下移者24例，单纯三尖瓣后叶发生下移者12例，三尖
瓣隔叶与后叶同时下移者122例，三尖瓣前叶病变（下移、发育异常、与室壁黏连等）者105例，其中心功
能Ⅳ级隔叶和后叶下移距离最大，三尖瓣重度反流人数最多，三尖瓣口流速和压差均最大；心功能Ⅲ级、
Ⅳ级患者固有右房、房化右室、功能右室左右及上下径各超声数值都较高，右心各房室径变化较大，固有
右房、房化右室与功能右室上下径的比值结果也最大，患者的左室射血分数随着年龄的增长呈现减低趋
势。结论 不同心功能分组的成人EA患者有独特临床及超声特点。随着年龄的增长，左心功能呈现降低趋
势，临床应对此类患者做到早发现、早诊断、早治疗，以提高患者预后。

Objective To analyze the clinical and ultrasound features in adult patients with Ebstein anomaly (EA), and provide for clinicians with more valuable information about the disease. Methods The clinical and ultrasound materials were retrospectively analyzed in EA patients (n=168) chosen from Beijing Anzhen Hospital affiliated to Capital Medical University from Jan. 2002 to Aug. 2018. Results A total of 168 EA patients were enrolled, among them there were 127 patients (75.60%) with intracardiac or extracardiac diseases. The enrolled patients mostly received surgical treatment for tricuspid valve malformations and related complicated diseases, and 78 patients (46.43%) underwent EA corrective surgery, and none of the patients with grade IV of NYHA had undergone surgery. There were 36 patients were hospitalized due to arrhythmia or complicated arrhythmia, and there were 3 cases died (1.79%), who were all ones with grade III of NYHA. There were 24 EA patients with simple tricuspid septum descending, 12 with posterior tricuspid leaflet descending, and 122 with simultaneous descending of tricuspid septum and posterior tricuspid leaflet. There are 105 patients with lesions of anterior tricuspid leaflet (descent, abnormal development, adhesion with ventricular wall, etc.). The descending distance of tricuspid septum and posterior tricuspid leaflet was the longest, the number of patients with severe tricuspid refluxwas the largest, and tricuspid orifice flow velocity and pressure difference were the highest in patients with grade IV of NYHA. The ultrasonic numerical value of left and right and upper and lower diameters of intrinsic right atrium, atrialized right ventricle and functional right ventricle were all higher, the atrial and ventricular diameters of right heart had greater changes, ratio results of upper and lower diameters of intrinsic right atrium, atrialized right ventricle and functional right ventricle were the largest, and left ventricular ejection fraction (LVEF) showed a descending trend with age increasing in patients with grade III and grade IV of NYHA. Conclusion Adult EA patients with different cardiac function grades have their own clinical and ultrasound features. As patient’s age increases, their left heart function shows a descending trend. Clinicians should give early detection, early diagnosis and early treatment to these patients to improve the prognosis.

北京市医院管理局“登峰”计划专项经费资助
(DFL20180602)