Service de Médecine Interne et Maladies Orphelines, Centre Hospitalier Universitaire de Nancy, Hôpitaux de Brabois, and Faculté de Médecine, avenue de Forêt de Haye, 54500 Vandoeuvre-Cedex, France.
Int J Cardiol. 2013 Jan 20;162(3):172-8. doi: 10.1016/j.ijcard.2011.05.070. Epub 2011 Jun 2.
The aim of the study was to identify, in addition to conduction defects, possible predictors of cardiac events and death in patients with myotonic dystrophy (DM1).
A retrospective observational cohort study was undertaken. Baseline clinical and non-invasive cardiac and respiratory investigations were obtained from 107 DM1 patients, who were regularly re-examined. Primary end-points were occurrence of cardiac events (pacemaker implantation or tachyarrhythmia) or death. Probability of an event was calculated using the Kaplan-Meier method, while contributing factors were assessed using univariate and multivariate (Cox model) analyses.
Cardiac events occurred in 34 patients (29%). Age, muscular impairment, infantile onset, restrictive lung disease (RLD), ECG conduction defects, left ventricular ejection fraction (LVEF) below 50%, and arrhythmia detected during Holter monitoring were predictors of cardiac events. Multivariate analysis indicated that age, RLD, ECG conduction defects, Holter arrhythmia and LVEF remained independent predictors. Probability of cardiac events was 2.5% (5%CI: 0-7%) at 1 year and 6% (5%CI: 0-14%) at 3 years in patients younger than 42 years with normal ECG, Holter, LVEF and lung volumes. Advancing age, distal or proximal weakness and RLD characterized all non-survivors (n=14).
Cardiac events or death are predicted not only by conduction defects or cardiomyopathy in DM1, but also by RLD, muscular disability and advancing age. Addition of these criteria should modulate time intervals for patient follow-up examinations. In young patients with normal baseline investigations, screening investigations every 2 or 3 years seem to be sufficient.
本研究旨在除了传导缺陷外，还确定肌强直性营养不良（DM1）患者发生心脏事件和死亡的可能预测因素。
进行了一项回顾性观察队列研究。从 107 例 DM1 患者中获得了基线临床和非侵入性心脏和呼吸检查，并定期对其进行复查。主要终点为发生心脏事件（起搏器植入或心动过速）或死亡。使用 Kaplan-Meier 方法计算事件发生的概率，同时使用单变量和多变量（Cox 模型）分析评估相关因素。
34 例患者（29%）发生了心脏事件。年龄、肌肉功能障碍、婴儿期发病、限制性肺疾病（RLD）、心电图传导缺陷、左心室射血分数（LVEF）<50%以及 Holter 监测时发现的心律失常是心脏事件的预测因素。多变量分析表明，年龄、RLD、心电图传导缺陷、Holter 心律失常和 LVEF 仍然是独立的预测因素。在年龄小于 42 岁、心电图、Holter、LVEF 和肺容积正常的患者中，心脏事件的概率为 1 年时为 2.5%（5%CI：0-7%），3 年时为 6%（5%CI：0-14%）。所有非幸存者（n=14）均表现出进展性年龄、远端或近端无力和 RLD。
DM1 不仅通过传导缺陷或心肌病预测心脏事件或死亡，还通过 RLD、肌肉功能障碍和年龄增长来预测。添加这些标准应调节患者随访检查的时间间隔。在基线检查正常的年轻患者中，每 2 或 3 年进行一次筛查检查似乎就足够了。
