Department of Cardiovascular Disease, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA.
Department of Internal Medicine, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA.
Eur Heart J. 2018 Jun 1;39(21):1970-1977a. doi: 10.1093/eurheartj/ehx794.
Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA.
All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed. Clinical variables, cardiovascular surgical procedure(s), and cause of death were recorded. Sudden death incidence from birth and following tricuspid valve (TV) surgery were estimated using the Kaplan-Meier method. Cox regression analysis was used to identify clinical and surgical predictors of SD. The cohort comprised of 968 patients [mean age 25.3 years, 41.5% male; 79.8% severe EA, 18.6% accessory pathway, 0.74% implantable cardioverter-defibrillator (ICD) placement]. The 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%, respectively. Prior ventricular tachycardia [hazard ratio (HR) 6.37, P < 0.001)], heart failure (HR 5.64, P < 0.001), TV surgery (HR 5.94, P < 0.001), syncope (HR 2.03, P = 0.019), pulmonic stenosis (HR 3.42, P = 0.001), and haemoglobin > 15 g/dL (HR 2.05, P = 0.026) were multivariable predictors of SD. In a similar subgroup analysis of patients who underwent TV surgery, all of the above factors except syncope were significantly associated with post-operative SD on multivariable analysis.
Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.
左、右心室功能障碍或结构改变是导致心源性猝死（SD）的一个明确的危险因素。Ebstein 畸形（EA）可同时存在左右心异常；然而，SD 的预测因素尚未被描述。因此，我们试图在一个大型 EA 患者队列中描述 SD 的发生率和危险因素。
回顾性分析了 40 年来在一家高容量机构接受评估的所有 EA 患者。记录了临床变量、心血管手术和死因。使用 Kaplan-Meier 方法估计了从出生到接受三尖瓣（TV）手术后 SD 的发生率。Cox 回归分析用于识别 SD 的临床和手术预测因素。该队列包括 968 例患者[平均年龄 25.3 岁，41.5%为男性；79.8%为严重 EA，18.6%为旁路，0.74%为植入式心脏复律除颤器（ICD）放置]。从出生起，SD 的 10 年、50 年和 70 年累积发生率分别为 0.8%、8.3%和 14.6%。先前的室性心动过速[危险比（HR）6.37，P<0.001]、心力衰竭（HR 5.64，P<0.001）、TV 手术（HR 5.94，P<0.001）、晕厥（HR 2.03，P=0.019）、肺动脉瓣狭窄（HR 3.42，P=0.001）和血红蛋白>15g/dL（HR 2.05，P=0.026）是 SD 的多变量预测因素。在 TV 手术后患者的类似亚组分析中，除晕厥外，所有上述因素在多变量分析中均与术后 SD 显著相关。
EA 患者发生 SD 的风险显著增加。确定的关键临床 SD 预测因素可有助于风险分层，并可能指导原发性预防 ICD 植入。
