AP-HP, Groupe Hospitalier Pitié-Salpêtrière, L'institut de Cardiométabolisme et Nutrition, Département de Cardiologie, Paris, France; Centre de Référence des Maladies Cardiaques Héréditaires, Paris, France.
AP-HP, Groupe Hospitalier Pitié-Salpêtrière, L'institut de Cardiométabolisme et Nutrition, Département de Cardiologie, Paris, France.
JACC Clin Electrophysiol. 2018 Jun;4(6):757-768. doi: 10.1016/j.jacep.2018.04.017.
The purpose of this study was to identify clinical factors associated with arrhythmic events and sudden cardiac death (SCD), and to evaluate the prognostic value of electrophysiological study (EPS) in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients without implantable cardioverter-defibrillators (ICDs).
ARVC/D is an inherited cardiomyopathy characterized by a risk of SCD. Few studies have evaluated predictive factors of ventricular arrhythmias (VAs) in patients without ICDs.
Between 2000 and 2010, all consecutive patients with ARVC/D without ICDs and with EPS at diagnosis were enrolled. Patients that received an ICD during follow-up were censored at the date of implantation, and in that case, only VAs that occurred before ICD implantation were analyzed. Risk factors for any VA event were determined by Cox regression. Patients that only experienced SCD or aborted cardiac arrest (ACA) were reported.
A total of 137 consecutive patients (78% male) diagnosed with ARVC/D without ICD were enrolled. 31% had sustained ventricular tachycardia at diagnosis. After mean follow-up of 42 ± 31 months, 19 patients experienced an episode of sustained VA and 5 patients experienced a SCD/ACA. No event occurred in asymptomatic patients. Left ventricular ejection fraction ≤50% (p = 0.024), positive EPS (p = 0.017), and physical activity >6 h/week (p = 0.025) were independently associated with occurrence of VAs. SCD/ACA exclusively occurred in male probands with definite diagnosis and syncope.
In this cohort of ARVC/D patients without ICD, left ventricular ejection fraction ≤50%, positive EPS, and physical activity >6 h/week were independent predictors of VAs, whereas asymptomatic patients at diagnosis were at low risk. EPS predicted all VAs but had limited value to predict SCD/ACA.
本研究旨在确定与心律失常事件和心脏性猝死（SCD）相关的临床因素，并评估在无植入式心律转复除颤器（ICD）的致心律失常性右心室心肌病/发育不良（ARVC/D）患者中电生理研究（EPS）的预后价值。
ARVC/D 是一种遗传性心肌病，其 SCD 风险较高。很少有研究评估无 ICD 的患者室性心律失常（VA）的预测因素。
在 2000 年至 2010 年间，所有连续诊断为 ARVC/D 且无 ICD 且在诊断时接受 EPS 的患者均被纳入研究。在随访期间接受 ICD 治疗的患者以植入日期为截止日期，在此情况下，仅分析植入 ICD 前发生的 VA。通过 Cox 回归确定任何 VA 事件的危险因素。仅报告 SCD 或心搏骤停（ACA）患者。
共纳入 137 例连续诊断为 ARVC/D 且无 ICD 的患者（78%为男性）。31%的患者在诊断时患有持续性室性心动过速。平均随访 42±31 个月后，19 例患者发生持续性 VA 发作，5 例患者发生 SCD/ACA。无症状患者未发生任何事件。左心室射血分数≤50%（p=0.024）、EPS 阳性（p=0.017）和每周体力活动>6 小时（p=0.025）与 VA 的发生独立相关。SCD/ACA 仅发生在具有明确诊断和晕厥的男性先证者中。
在本队列中，无 ICD 的 ARVC/D 患者中，左心室射血分数≤50%、EPS 阳性和每周体力活动>6 小时是 VA 的独立预测因素，而诊断时无症状的患者风险较低。EPS 预测了所有 VA，但对预测 SCD/ACA 的价值有限。
