Abstract
For the management of myotonic dystrophy, cardiac involvements should be treated as being important as same as the treatment of respiratory failure at the aspect of prognosis particularly in patients with myotonic dystrophy type 1 (DM1). The most critical cardiac involvement is cardiac conduction diseases including sick sinus syndrome and AV block to be cared with cardiac pacemakers. The progressive cardiac conduction disease has been reported to be associated with sudden death in DM1 patients, and physicians need to manage when cardiac devices should be implanted in DM1 patients on the results by noninvasive or invasive cardiac tests, e.g., electrocardiogram, 24-h Holter monitoring, or electrophysiological study. Ventricular tachyarrhythmias should be also monitored for DM1 patients, and implanted cardioverter defibrillator but not pacemakers may be suitable especially for DM1 patients with severe conduction diseases. Atrial fibrillation has been reported to be common and a clinical marker to be associated with sudden death in DM1 patients. We regularly need to care whether these arrhythmias appear on electrocardiograms. As shown in the recent guideline in American Heart Association, neurologists are required to care DM1 patients with cardiologists.
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Abbreviations
- ACEI:
-
Angiotensin-converting enzyme inhibitor
- ARB:
-
Angiotensin receptor blocker
- AV:
-
Atrioventricular
- CRT:
-
Cardiac resynchronization therapy
- DM:
-
Myotonic dystrophy
- DM1:
-
Myotonic dystrophy type 1
- DM2:
-
Myotonic dystrophy type 2
- DMPK:
-
Myotonin-protein kinase
- ICD:
-
Implantable cardioverter defibrillator
- LVEF:
-
Left ventricular ejection fraction
- MMRGlu:
-
Myocardial metabolic rate for glucose
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Acknowledgment
Authors thank Drs. Kazuhiko Segawa, Satoshi Kuru, Shugo Suwazono, Shingo Sasaki, Minoru Horie, Tsuyoshi Matsumura, and Masanori Takahashi for clinical data and valuable comments. This work was partly supported by grants from the Ministry of Health, Labour and Welfare of Japan (H28-Nanchitou(Nan)-Ippan-030) and Japan Agency for Medical Research and Development (AMED) (17ek0109259).
Disclosures. None.
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Itoh, H., Tamura, T. (2018). Cardiac Involvements in Myotonic Dystrophy. In: Takahashi, M., Matsumura, T. (eds) Myotonic Dystrophy. Springer, Singapore. https://doi.org/10.1007/978-981-13-0508-5_4
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