Combined cardiomyopathy and skeletal myopathy: a variant with atrial fibrillation and ventricular tachycardia.

Vlay SC; Vlay LC; Coyle PK

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Combined cardiomyopathy and skeletal myopathy: a variant with atrial fibrillation and ventricular tachycardia.

机译：合并心肌病和骨骼肌病：心房颤动和室性心动过速的变异。

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This article describes a family characterized by combined cardiomyopathy and nonspecific skeletal myopathy who present in the third to fifth decades with cardiac manifestations but earlier have evidence of subtle skeletal muscle dysfunction. They differ from previously defined syndromes and potentially represent a different genetic expression or mutation. Cardiomyopathy presents with atrial arrhythmias including AF and atrial flutter. Life-threatening ventricular tachyarrhythmias occur next with onset of ventricular dysfunction. Electrophysiological study revealed sustained monomorphic VT. Affected family members benefitted from an ICD and progression to congestive heart failure (CHF) occurred late. Skeletal myopathy continues with marked progressive muscle weakness and inability to ambulate without assistance. Genetic analysis is currently ongoing. Neurological evaluation in all three family members revealed nonspecific myopathy affecting the psoas and iliopsoas muscles. Atrophy and wasting of the facial and temporalis muscles were common. Skeletal muscle biopsy revealed myofiber atrophy consistent with myopathy.

机译：本文介绍了一个以心肌病和非特异性骨骼肌病合并症为特征的家庭，他们在第三到第五个十年中出现心脏表现，但更早的时候就出现了骨骼肌功能障碍的证据。它们不同于先前定义的综合症，并可能代表不同的基因表达或突变。心肌病表现为房颤，包括房颤和房扑。危及生命的室性快速性心律失常继发于心室功能障碍。电生理研究显示持续单形性室速。患病家庭成员受益于ICD，并且进展为充血性心力衰竭（CHF）较晚。骨骼肌病继续伴有明显的进行性肌无力和无助下不能走动。遗传分析目前正在进行中。所有三个家庭成员的神经系统评价均显示非特异性肌病，影响腰肌和and肌。面部和颞侧肌肉萎缩和消瘦很常见。骨骼肌活检显示肌纤维萎缩与肌病一致。

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《PACE: Pacing and clinical electrophysiology》 |2001年第1期|共9页
作者
Vlay SC; Vlay LC; Coyle PK;
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正文语种 eng
中图分类诊断学;
关键词
Atrial Fibrillation; Muscular Dystrophies; Myocardial Diseases; Tachycardia; Ventricular; 心房颤动; 心肌疾病; 心动过速; 室性;

机译：Atrial Fibrillation;Muscular Dystrophies;Myocardial Diseases;Tachycardia;Ventricular;心房颤动;心肌疾病;心动过速;室性;

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1. Combined cardiomyopathy and skeletal myopathy: a variant with atrial fibrillation and ventricular tachycardia. [J] . Vlay SC, Vlay LC, Coyle PK PACE: Pacing and clinical electrophysiology . 2001,第9aPta1期

机译：合并心肌病和骨骼肌病：心房颤动和室性心动过速的变异。
2. Atrial arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and ventricular tachycardia. [J] . Chu AF, Zado E, Marchlinski FE The American Journal of Cardiology . 2010,第5期

机译：心律失常性右室心肌病/发育异常和室性心动过速患者的房性心律失常。
3. Efficacy of Combined Cox-Maze IV and Ventricular Septal Myectomy for Treatment of Atrial Fibrillation in Patients With Obstructive Hypertrophic Cardiomyopathy [J] . Boll Griffin, Rowin Ethan J., Maron Barry J., The American Journal of Cardiology . 2020,第1期

机译：Cox-maze IV和心室隔膜组合疗效治疗阻塞性肥厚性心肌病患者心房颤动的疗效
4. Refined Ventricular Activity Cancellation in Electrograms During Atrial Fibrillation by Combining Average Beat Subtraction and Interpolation [C] . Massimo W. Rivolta, Roberto Sassi, Muhamed Vila Annual International Conference of the IEEE Engineering in Medicine and Biology Society . 2019

机译：结合平均搏动减法和内插法在心房颤动期间在心电图中精细的心室活动抵消
5. DNA mutation analysis of voltage-gated ion channel genes in two families with sick sinus syndrome and atrial fibrillation/AV nodal reentry tachycardia. [D] . Oh, Frances. 2010

机译：两个病态窦房结综合征和房颤/房室结折返性心动过速患者的电压门控离子通道基因的DNA突变分析。
6. Editorial to The differences of atrial thrombus locations and variable response to anticoagulation in non‐valvular atrial fibrillation with ventricular cardiomyopathy [O] . Wei‐Hsin Chung, Hung‐Pin Wu, Jan‐Yow Chen, 2020

机译：编辑措施心室心肌病变非瓣膜心房颤动中的心房血栓位置和变性对抗凝的差异
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Combined cardiomyopathy and skeletal myopathy: a variant with atrial fibrillation and ventricular tachycardia.

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