Prolonged Corrected QT Interval in Patients with Myotonic Dystrophy Type 1
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background and Purpose Sudden cardiac death is one of the leading causes of death in patients with myotonic dystrophy type 1 (DM1). It has been proposed that a prolonged QT interval is associated with sudden cardiac death in several neurological diseases, including multiple system atrophy, idiopathic Parkinson’s disease, and diabetic autonomic neuropathy. However, analyses of the corrected QT (QTc) interval in DM1 patients are rare in the literature. The purposes of this study were to determine the association between the QT interval and DM1, and the affecting factors. Methods Thirty-nine patients diagnosed with DM1 through genetic testing were enrolled. The QTc interval (calculated using Bazett’s formula: QTc=QT/√RR) was compared between these patients and 39 normal healthy controls. The clinical and laboratory factors affecting QTc interval in the patient group were investigated. Results The QTc interval was significantly longer in the DM1 group (411.2±44.7 msec, mean±SD) than in the normal control group (355.6±20.6 msec). Intragroup analysis revealed that a prolonged QTc interval in DM1 patients was associated with being female and older, having a longer disease duration, and exhibiting abnormal electrocardiography findings. Conclusions The higher incidence of sudden cardiac death in the DM1 population is associated with the observed prolonged QTc interval in those patients.
Abstract
Background and Purpose Sudden cardiac death is one of the leading causes of death in patients with myotonic dystrophy type 1 (DM1). It has been proposed that a prolonged QT interval is associated with sudden cardiac death in several neurological diseases, including multiple system atrophy, idiopathic Parkinson’s disease, and diabetic autonomic neuropathy. However, analyses of the corrected QT (QTc) interval in DM1 patients are rare in the literature. The purposes of this study were to determine the association between the QT interval and DM1, and the affecting factors. Methods Thirty-nine patients diagnosed with DM1 through genetic testing were enrolled. The QTc interval (calculated using Bazett’s formula: QTc=QT/√RR) was compared between these patients and 39 normal healthy controls. The clinical and laboratory factors affecting QTc interval in the patient group were investigated. Results The QTc interval was significantly longer in the DM1 group (411.2±44.7 msec, mean±SD) than in the normal control group (355.6±20.6 msec). Intragroup analysis revealed that a prolonged QTc interval in DM1 patients was associated with being female and older, having a longer disease duration, and exhibiting abnormal electrocardiography findings. Conclusions The higher incidence of sudden cardiac death in the DM1 population is associated with the observed prolonged QTc interval in those patients.
참고문헌 (35)
- Gharehbaghi-Schnell EB, "Genotype-phenotype correlation in myotonic dystrophy", Clinical Genetics, 53, pp.20~26 (1998)
- Pizzuti A, "The myotonic dystrophy gene", Archives of neurology, 50, pp.1173~1179 (1993)
- Jaspert A, "Myotonic dystrophy: correlation of clinical symptoms with the size of the CTG trinucleotide repeat", Journal of Neurology, 242, pp.99~104 (1995)
- Mondelli M, "Axonal motor and sensory neuropathy in myotonic dystrophy", Acta Neurologica Scandinavica, 88, pp.141~148 (1993)
- Meola G., "Clinical and genetic heterogeneity in myotonic dystrophies", Muscle & Nerve, 23, pp.1789~1799 (2000)
- Harley HG, "Expansion of an unstable DNA region and phenotypic variation in myotonic dystrophy", Nature, 355, pp.545~546 (1992)
- Aslanidis C, "Cloning of the essential myotonic dystrophy region and mapping of the putative defect", Nature, 355, pp.548~551 (1992)
- Harley HG, "Size of the unstable CTG repeat sequence in relation to phenotype and parental transmission in myotonic dystrophy", The American Journal of Human Genetics, 52, pp.1164~1174 (1993)
- Ashizawa T, "Anticipation in myotonic dystrophy. I. Statistical verification based on clinical and haplotype findings", Neurology, 42, pp.1871~1877 (1992)
- Melacini P, "Correlation between cardiac involvement and CTG trinucleotide repeat length in myotonic dystrophy", Journal of the American College of Cardiology, 25, pp.239~245 (1995)
- Tokgozoglu LS, "Cardiac involvement in a large kindred with myotonic dystrophy. Quantitative assessment and relation to size of CTG repeat expansion", JAMA, 274, pp.813~819 (1995)
- Finsterer J, "Relation of cardiac abnormalities and CTG-repeat size in myotonic dystrophy", Clinical Genetics, 59, pp.350~355 (2001)
- Sabovic M, "Relation of CTG expansion and clinical variables to electrocardiogram conduction abnormalities and sudden death in patients with myotonic dystrophy", Neuromuscular Disorders, 13, pp.822~826 (2003)
- Pelargonio G, "Myotonic dystrophy and the heart", Heart, 88, pp.665~670 (2002)
- Mathieu J, "A 10-year study of mortality in a cohort of patients with myotonic dystrophy", Neurology, 52, pp.1658~1662 (1999)
- Glickstein JS, "Abnormalities of the corrected QT interval in familial dysautonomia: an indicator of autonomic dysfunction", Journal of Pediatrics, 122, pp.925~928 (1993)
- Lo SS, "QT interval and dispersion in primary autonomic failure", Heart, 75, pp.498~501 (1996)
- Deguchi K, "Abnormalities of rate-corrected QT intervals in Parkinson’s disease- a comparison with multiple system atrophy and progressive supranuclear palsy", Journal of the Neurological Sciences, 199, pp.31~37 (2002)
- Breton R, "Usefulness of clinical and electrocardiographic data for predicting adverse cardiac events in patients with myotonic dystrophy", Canadian Journal of Cardiology, 25, pp.e23~e27 (2009)
- Groh WJ, "Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1", The New England Journal of Medicine, 358, pp.2688~2697 (2008)
- Magrì D, "Increased temporal dispersion of myocardial repolarization in myotonic dystrophy type 1: beyond the cardiac conduction system", International Journal of Cardiology, 156, pp.259~264 (2012)
- Mathieu J, "Myotonic dystrophy: clinical assessment of muscular disability in an isolated population with presumed homogeneous mutation", Neurology, 42, pp.203~208 (1992)
- Hannan PJ, "Concerning the units for the QT interval corrected by Bazett’s formula", Circulation, 96, pp.3799~ (1997)
- Melacini P, "The natural history of cardiac involvement in myotonic dystrophy: an eight-year follow-up in 17 patients", Clinical Cardiology, 11, pp.231~238 (1988)
- Hayashi Y, "Cardiac abnormalities and cytosine-thymine-guanine trinucleotide repeats in myotonic dystrophy", American Heart Journal, 134(2 Pt 1), pp.292~297 (1997)
- Diedrich A, "Modulation of QT interval during autonomic nervous system blockade in humans", Circulation, 106, pp.2238~2243 (2002)
- Di Leo R, "Cardiovascular autonomic control in myotonic dystrophy type 1: a correlative study with clinical and genetic data", Neuromuscular Disorders, 14, pp.136~141 (2004)
- Olofsson BO, "Assessment of autonomic nerve function in myotonic dystrophy", Journal of the Autonomic Nervous System, 29, pp.187~192 (1990)
- Pierangeli G, "Autonomic nervous system function in myotonic dystrophy", The Italian Journal of Neurological Sciences, 13, pp.589~592 (1992)
- Inoue K, "Assessment of autonomic function in myotonic dystrophy by spectral analysis of heart-rate variability", Journal of the Autonomic Nervous System, 55, pp.131~134 (1995)
- Rana BS, "Relation of QT interval dispersion to the number of different cardiac abnormalities in diabetes mellitus", American Journal of Cardiology, 90, pp.483~487 (2002)
- Behr E, "Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome", The Lancet, 362, pp.1457~1459 (2003)
- Rautaharju PM, "Usefulness of T-axis deviation as an independent risk indicator for incident cardiac events in older men and women free from coronary heart disease (the Cardiovascular Health Study)", American Journal of Cardiology, 88, pp.118~123 (2001)
- Phillips MF, "Cardiac disease in myotonic dystrophy", Cardiovascular Research, 33, pp.13~22 (1997)
- Bhakta D, "Prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I", American Heart Journal, 147, pp.224~227 (2004)