Garcia Rodrigue, Rehman Michaela, Goujeau Cyril, Degand Bruno, Le Gal François, Stordeur Benjamin, Labarre Quentin, Christiaens Luc, Bouleti Claire
CHU Poitiers, Service de Cardiologie, 2 rue de la Milétrie, F-86021 Poitiers, France.
CHU Poitiers, Service de Cardiologie, 2 rue de la Milétrie, F-86021 Poitiers, France.
Int J Cardiol. 2017 Sep 15;243:424-430. doi: 10.1016/j.ijcard.2017.05.061. Epub 2017 May 19.
Type 1 myotonic dystrophy (DM1) patients' prognosis is very poor. Up until now, only a few prognostic factors for cardiovascular events have been identified, and they are predictive of end-stage disease. The aim was to assess the prognostic value of global longitudinal strain (GLS) for cardiovascular events in asymptomatic DM1 patients.
DM1 patients were included between 2011 and 2015 and followed up until January 2016. Patients underwent a transthoracic echocardiography at inclusion. The primary endpoint was a composite of all-cause mortality, type 2 Mobitz 2 and type 3 atrioventricular block, symptomatic sino-atrial block, HV interval≥70ms at invasive electrophysiology exploration, left ventricular ejection fraction (LVEF) ≤45% and newly developed atrial fibrillation.
Forty-six patients (25 males, mean age 40years old) were included. The primary outcome was reached in 14 patients with a mean follow-up of 38months. GLS of patients who reached the primary endpoint was significantly impaired as compared to those who did not (-15.1 [-16.7; -12.7] vs. -18.2 [-19.2; -16.7] respectively; P=0.001). According to ROC curve analysis, probability of primary outcome occurrence was significantly greater in patients with GLS values≥-17.2% (P=0.001). On multivariate analysis, PR electrocardiogram interval and GLS remained significantly and independently associated with the primary endpoint [hazard ratio (HR) 1.03, 95% confidence interval (CI) 1.01-1.04, P=0.006 for PR interval; HR 1.4, 95% CI 1.1-1.7, P=0.002 for GLS] while LVEF alone was not.
Left ventricular GLS is a powerful marker to predict cardiovascular events in asymptomatic DM1 patients, independently of LVEF.
1型强直性肌营养不良（DM1）患者的预后很差。到目前为止，仅确定了少数心血管事件的预后因素，且它们只能预测疾病终末期。目的是评估整体纵向应变（GLS）对无症状DM1患者心血管事件的预后价值。
纳入2011年至2015年间的DM1患者，并随访至2016年1月。患者在纳入时接受经胸超声心动图检查。主要终点是全因死亡率、2型莫氏Ⅱ度和3度房室传导阻滞、有症状的窦房阻滞、侵入性电生理检查时HV间期≥70ms、左心室射血分数（LVEF）≤45%以及新发生的心房颤动的复合终点。
纳入46例患者（25例男性，平均年龄40岁）。平均随访38个月后，14例患者达到主要终点。达到主要终点的患者的GLS与未达到主要终点的患者相比明显受损（分别为-15.1[-16.7；-12.7]和-18.2[-19.2；-16.7]；P=0.001）。根据ROC曲线分析，GLS值≥-17.2%的患者发生主要终点的概率显著更高（P=0.001）。多因素分析显示，PR心电图间期和GLS仍与主要终点显著且独立相关[PR间期的风险比（HR）为1.03，95%置信区间（CI）为1.01-1.04，P=0.006；GLS的HR为1.4，95%CI为1.1-1.7，P=0.002]，而单独的LVEF并非如此。
左心室GLS是预测无症状DM1患者心血管事件的有力指标，独立于LVEF。
