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

ITOH Hideki 30402738 0000-0002-5104-3313 HISAMATSU Takashi TAMURA Takuhisa SEGAWA Kazuhiko TAKAHASHI Toshiaki TAKADA Hiroto KURU Satoshi WADA Chizu SUZUKI Mikiya SUWAZONO Shugo SASAKI Shingo OKUMURA Ken HORIE Minoru 90183938 0000-0002-9029-2339 TAKAHASHI Masanori P MATUMURA Tsuyoshi 滋賀医科大学

2020.09

概要

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.

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参考文献

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SUPPLEMENTAL MATERIAL

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Figure S1. Multivariate analysis between the characteristics of patients and outcomes.

Downloaded from http://ahajournals.org by on September 18, 2020

Multivariate relative risks and P-values were calculated using the Cox proportional-hazards

model for time to death from any cause, death from progressive neuromuscular respiratory

failure, sudden death, and incidence of cardiac events. The multivariate models were limited to

variables emerging with a P<0.1 from the univariate analysis. All covariates, except for age,

were time-dependent. P-values: *<0.05; †<0.01; ‡<0.001. §Hazard ratio per 1-year older. ¶Hazard

ratio per 10 bpm increase in heart rate. HR, hazard ratio; NA, not available; AF, atrial

fibrillation; VT, ventricular fibrillation.

Downloaded from http://ahajournals.org by on September 18, 2020

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