Bienias Piotr, Łusakowska Anna, Ciurzyński Michał, Rymarczyk Zuzanna, Irzyk Katarzyna, Kurnicka Katarzyna, Kamińska Anna, Pruszczyk Piotr
Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.
Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
Pacing Clin Electrophysiol. 2016 Sep;39(9):959-68. doi: 10.1111/pace.12924. Epub 2016 Aug 18.
Patients with myotonic muscular dystrophy (dystrophia myotonica, DM) are at risk of sudden cardiac death due to diverse arrhythmias, especially progressive atrioventricular (AV) conduction abnormalities. However, there are limited data on supraventricular and potentially life-threatening ventricular arrhythmias, especially according to type 1 and type 2 DM.
A group of 94 unselected consecutive patients with genetically confirmed DM and 45 healthy controls underwent electrocardiography, echocardiography, and 24-hour Holter monitoring. DM1 was diagnosed in 51, while DM2 in 43 patients (with mean age of 37.3 ± 12.5 and 48.3 ± 13.3 years, respectively).
DM1 subjects presented more frequently intraventricular conduction defects (29.4% vs 6.6%, P = 0.0003) and first-degree AV block (25.0% vs 4.6%, P = 0.008) than DM2 patients. Nonsustained supraventricular tachycardia (37.2% vs 3.8%, P = 0.001) and nonsustained ventricular tachycardia and/or R-on-T ventricular beats (23.2% vs 7.8%, P = 0.04) were more frequently observed in DM2 than in DM1. No relationship between disease duration and neurological status and occurrence of arrhythmias was observed. Multivariate analysis showed that independent predictor for bradyarrhythmias occurrence was DM1 only (odds ratio [OR] 6.4, 95% confidence interval [CI] 2.0-20.8, P = 0.002), while for supraventricular or ventricular arrhythmias occurrence it was DM2 (OR 4.1, 95% CI 1.5-11.4, P = 0.007) and increased age (OR 1.09, 95% CI 1.05-1.15, P < 0.0001).
In the relatively large groups of DM1 and DM2 patients, we observed frequent various arrhythmias, which warrant their close cardiac monitoring. DM1 subjects when compared to DM2 presented more frequently intraventricular and AV conduction defects. However, all types of tachyarrhythmias (except atrial fibrillation) were more frequently observed in DM2 patients.
强直性肌营养不良（DM）患者因多种心律失常，尤其是进行性房室传导异常，存在心源性猝死风险。然而，关于室上性心律失常和潜在危及生命的室性心律失常的数据有限，尤其是根据1型和2型DM的情况。
一组94例未经选择的连续基因确诊的DM患者和45例健康对照者接受了心电图、超声心动图和24小时动态心电图监测。51例诊断为DM1，43例诊断为DM2（平均年龄分别为37.3±12.5岁和48.3±13.3岁）。
与DM2患者相比，DM1患者更频繁出现室内传导阻滞（29.4%对6.6%，P=0.0003）和一度房室传导阻滞（25.0%对4.6%，P=0.008）。DM2患者比DM1患者更频繁观察到非持续性室上性心动过速（37.2%对3.8%，P=0.001）以及非持续性室性心动过速和/或R波落在T波上的室性早搏（23.2%对7.8%，P=0.04）。未观察到病程和神经状态与心律失常发生之间的关系。多因素分析显示，缓慢性心律失常发生的独立预测因素仅为DM1（比值比[OR]6.4，95%置信区间[CI]2.0-20.8，P=0.002），而室上性或室性心律失常发生的独立预测因素为DM2（OR 4.1，95%CI 1.5-11.4，P=0.007）和年龄增加（OR 1.09，95%CI 1.05-1.15，P<0.0001）。
在相对较大的DM1和DM2患者群体中，我们观察到频繁出现各种心律失常，这需要对他们进行密切的心脏监测。与DM2相比，DM1患者更频繁出现室内和房室传导阻滞。然而，DM2患者更频繁观察到所有类型的快速性心律失常（心房颤动除外）。
