Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

Ken Okumura¹  Shingo Sasaki²  Kazuhiko Segawa³  Hideki Itoh⁴  Minoru Horie⁴  Masanori P. Takahashi⁵  Chizu Wada⁶  Hiroto Takada⁷  Takuhisa Tamura⁸  Mikiya Suzuki⁸  Tsuyoshi Matumura⁹  Toshiaki Takahashi^1,10  Satoshi Kuru^1,11  Takashi Hisamatsu^1,12  Shugo Suwazono^1,13 

[1] Advanced Arrhythmia Therapeutic Branch Division of Cardiology Saiseikai Kumamoto Hospital Cardiovascular Center Kumamoto Japan;Department of Advanced Management of Cardiac Arrhythmias Hirosaki University Graduate School of Medicine Hirosaki Japan;Department of Cardiology National Center Hospital National Center of Neurology and Psychiatry Tokyo Japan;Department of Cardiovascular Medicine Shiga University of Medical Science Otsu Japan;Department of Functional Diagnostic Science Osaka University Graduate School of Medicine Suita Japan;Department of Neurology National Hospital Organization Akita National Hospital Akita Japan;Department of Neurology National Hospital Organization Aomori National Hospital Aomori Japan;Department of Neurology National Hospital Organization Higashisaitama National Hospital Saitama Japan;Department of Neurology National Hospital Organization Osaka Toneyama Medical Center 5‐1‐1 Toyonaka Japan;Department of Neurology National Hospital Organization Sendai‐Nishitaga Hospital Sendai Japan;Department of Neurology National Hospital Organization Suzuka National Hospital Suzuka Japan;Department of Public Health Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan;Division of Neurology and Center for Clinical Neuroscience National Hospital Organization Okinawa National Hospital Ginowan Japan;

关键词: conduction disturbance;    myotonic dystrophy;    sudden death;    ventricular tachycardia;   

DOI  :  10.1161/JAHA.119.015709

来源: DOAJ

【 摘 要 】

Background Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. Methods and Results This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine‐thymine‐guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow‐up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow‐up period of 87 months (Q1–Q3, 37–138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22–15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9–7.19, P<0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62–33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Conclusions Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.

【 授权许可】

Unknown