Pornprasertchai Varisara, Vijarnsorn Chodchanok, Kanjanauthai Supaluck, Chungsomprasong Paweena, Chanthong Prakul, Durongpisitkul Kritvikrom, Soongswang Jarupim
Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Congenit Heart Dis. 2019 Jul;14(4):619-627. doi: 10.1111/chd.12759. Epub 2019 Feb 21.
The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival.
To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era.
All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks.
A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis.
Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.
存活至成年期的埃布斯坦畸形（EA）患者数量不断增加，这意味着针对这种复杂病变的治疗方法有所改进。我们重新审视了EA患者的临床结局，以证明他们在“现实世界”中的生存率。
确定当代接受药物或手术治疗的EA患者的生存率及死亡预测因素。
对1994年至2016年间所有患有房室一致的EA患者进行回顾性研究。探究基线特征、初始超声心动图检查结果、治疗方法及结局。生存分析于2017年底进行。采用多变量分析评估死亡风险。
共分析了153例患者（年龄25.4±20.4岁，60%为女性）。其中，89例患者童年时被诊断为EA。在随访期间[中位时间5.2年（3天至23.5年）]，32例患者（20.9%）因严重心脏不良事件死亡。1年、5年和10年的总生存率分别为89%、82.2%和79%。在153例患者中，64例患者至少接受了一次手术干预[中位年龄17岁（1天至64.4岁）]。接受EA手术的患者1年、5年和10年的生存率分别为87.5%、82.4%和77.7%。这一生存率与89例未接受手术的EA患者的生存率相当：1年、5年和10年分别为89.9%、87.5%和81.8%。死亡的显著预测因素为：诊断时年龄≤2岁、三尖瓣（TV）z评分>3.80、TV移位>19.5mm/m、存在严重三尖瓣反流以及初始诊断时肺动脉瓣无正向血流。
在这个时代，EA患者的生存率中等良好。在本文中，我们报告了该患者群体中五个简单的死亡预测因素。
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