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目的:观察非手术治疗的成年三尖瓣下移畸形（EA）患者的远期预后，并初步探索其相关因素。方法:本研究为单中心回顾性研究，选取2004年3月至2008年12月于清华大学第一附属医院确诊的48例非手术治疗的成年EA患者。收集纳入患者的临床资料，并定期进行超声心动图、心电图和胸部X线检查，记录患者的生存情况。依据经胸超声心动图计算三尖瓣下移隔叶分隔比（SLAr），将病例分为3组：SLAr0.60组（9例）；依据胸部X线片计算胸心比（CTR）。使用Kaplan-Meier生存曲线计算患者的远期生存率，Cox比例风险回归模型分析死亡的相关因素。结果:本研究共纳入48例EA患者，男性19例，初次确诊年龄为（21.3±11.1）岁。42例患者（87.5%）合并心律失常，表现为预激综合征4例，室上性心动过速16例，右束支传导阻滞37例，心房颤动2例。随访时间为（148.8±16.8）个月，无失访，随访率为100%。随访期间死亡9例（18.8%）：心原性死亡6例（12.5%），其中心力衰竭3例，心律失常1例，猝死2例；意外事故死亡1例；不明原因死亡2例。随访期间SLAr0.60组的生存率分别为17/18、90.5%（19/21）及3/9。依据Kaplan-Meier生存曲线，3组的5年生存率分别为100%、100%和78%，10年生存率分别为94%、95%和44%。7例患者出现活动耐量下降、心力衰竭，其中SLAr>0.60组6例，0.45≤SLAr≤0.60组1例。2例患者发生脑血管栓塞事件。快速心律失常发作时间超过24 h者3例。单因素Cox回归分析结果显示，与SLAr0.60组的死亡风险更高（n HR=12.375，95%n CI 1.692~22.146，n P=0.015）；CTR≥0.65患者死亡风险是CTR0.60及CTR≥0.65与患者远期死亡相关。对于合并心律失常的EA患者，应积极建议药物或射频消融治疗心律失常。“,”Objective:To analyze the long-term outcome of unoperated Ebstein′s anomaly (EA) patients aged over 18 years, and to evaluate the related factor of outcomes.Methods:The data of 48 unoperated EA patients from March 2004 to December 2008 in the First Hospital of Tsinghua University, were analyzed. The clinical data of the patients were collected, and patients received regular echocardiography, ECG and chest X-ray examinations. Septal leaflet attachment ratio (SLAr) was calculated based on transthoracic echocardiography imagines. The patients were divided into 3 groups according to SLAr: SLAr0.60 (n n=9). Chest X-ray was used for measurement of cardiothoracic ratio (CTR). Kaplan Meier survival curve was used to calculate the long-term survival rate. Cox proportional hazards regression model was used to analyze the influencing factors of death.n Results:There were 19 males, and the mean age at diagnosis was (21.3±11.1) years. Forty-two patients (87.5%) were complicated with arrhythmia, including W-P-W syndrome (n n=4), supraventricular tachycardia (n n=16), right bundle branch block (n n=37), and atrial fibrillation (n n=2). The mean duration of follow-up was (148.8±16.8) months, the follow-up rate was 100% with no loss-to-follow up. Nine cases (18.8%) died during follow-up: 6 cases (12.5%) died of cardiac origin, including 3 cases of heart failure, 1 case of arrhythmia, and 2 cases of sudden death; 1 case died of accident; 2 cases died from unknown causes. During the follow-up period, the survival rates were 17/18, 19/21 (90.5%) and 3/9 in the SLAr0.60 group, respectively. According to Kaplan-Meier survival curve, the 5-year survival rates among the three groups were 100%, 100% and 78%, respectively. The 10-year survival rates among the three groups were 94%, 95% and 44%, respectively. Decreased activity tolerance and heart failure were found in 7 patients (6 patients in SLAr>0.60 group and 1 patient in 0.45≤SLAr≤0.60 group). Two patients had cerebrovascular embolism. There were 3 cases with tachyarrhythmia lasting more than 24 hours. Cox regression analysis showed that the risk of death was higher in patients with SLAr>0.60 than in patients with SLAr<0.45 (n HR=12.375, 95%n CI 1.692-22.146, n P=0.015); the risk of death in patients with CTR≥0.65 was 1.306 times higher than that in patients with CTR0.60 and CTR≥0.65 are risk factors of death. EA patients with arrhythmia should be actively treated with drugs or radiofrequency ablation.