Benhayon Daniel, Lugo Ricardo, Patel Rutuke, Carballeira Lidia, Elman Lauren, Cooper Joshua M
Electrophysiology Section, Cardiovascular Division, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
J Cardiovasc Electrophysiol. 2015 Mar;26(3):305-10. doi: 10.1111/jce.12604. Epub 2015 Feb 11.
Myotonic dystrophy (MD) is the most common muscular dystrophy in adults and is associated with sudden death. Reported predictors of sudden death in this population include atrial tachyarrhythmias, a PR interval greater than 240 milliseconds, aberrant QRS conduction, and any degree of AV block.
We sought to report on the arrhythmic outcome of a cohort of patients with a new diagnosis of genetically proven MD.
We performed a retrospective review of 37 patients with genetically confirmed MD referred to our electrophysiology clinic for primary cardiac screening.
There were 25 patients with MD type 1 (MD1) and 12 patients with MD type 2 (MD2). Eight patients with MD1 (32%) had atrial fibrillation, compared to only one patient with MD2 (8.3%). Patients with MD1 were more likely to have evidence of conduction disease abnormalities (40% vs. 8.3%, P = ns) and had a higher all-cause mortality (16% vs. 0%) than those with MD2. Criteria for recommending ICD implantation were based on sudden death risk factors suggested by published literature. Eleven patients were offered an ICD, 2 refused and died within the next year. Of the 9 patients who received an ICD, 8 had MD1. Three patients received appropriate shocks, 2 for monomorphic VT, and one for polymorphic VT.
The presence of AV conduction disturbance in MD patients is associated with a greater risk for ventricular arrhythmias. MD1 was more likely to be associated with cardiac arrhythmias than MD2. The incidence of ventricular arrhythmias among those who received a primary prevention ICD was 33% over 22 months, with 2 patients experiencing monomorphic VT and one experiencing polymorphic VT.
强直性肌营养不良（MD）是成人中最常见的肌营养不良症，与猝死有关。该人群中报道的猝死预测因素包括房性快速性心律失常、PR间期大于240毫秒、异常QRS传导以及任何程度的房室传导阻滞。
我们试图报告一组新诊断为基因确诊MD患者的心律失常结局。
我们对37例基因确诊MD并转诊至我们的电生理门诊进行原发性心脏筛查的患者进行了回顾性研究。
有25例1型MD（MD1）患者和12例2型MD（MD2）患者。8例MD1患者（32%）发生房颤，而MD2患者仅有1例（8.3%）。与MD2患者相比，MD1患者更有可能有传导疾病异常的证据（40%对8.3%，P=无显著差异），且全因死亡率更高（16%对0%）。推荐植入植入式心律转复除颤器（ICD）的标准基于已发表文献提出的猝死危险因素。11例患者被建议植入ICD，2例拒绝并在次年死亡。在9例接受ICD的患者中，8例为MD1。3例患者接受了适当的电击，2例因单形性室性心动过速，1例因多形性室性心动过速。
MD患者中房室传导障碍的存在与室性心律失常的风险增加有关。MD1比MD2更有可能与心律失常相关。在接受一级预防ICD的患者中，室性心律失常的发生率在22个月内为33%，2例患者发生单形性室性心动过速，1例发生多形性室性心动过速。
