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Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.
N Engl J Med. 2008 Jun 19; 358(25):2688-97.NEJM

Abstract

BACKGROUND

Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise.

METHODS

We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or third-degree atrioventricular block.

RESULTS

Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter-defibrillator during the follow-up period. During a mean follow-up period of 5.7 years, 81 patients died; there were 27 sudden deaths, 32 deaths from progressive neuromuscular respiratory failure, 5 nonsudden deaths from cardiac causes, and 17 deaths from other causes. Among the 17 patients who died suddenly in whom postcollapse rhythm was evaluated, a ventricular tachyarrhythmia was observed in 9. A severe ECG abnormality (relative risk, 3.30; 95% confidence interval [CI], 1.24 to 8.78) and a clinical diagnosis of atrial tachyarrhythmia (relative risk, 5.18; 95% CI, 2.28 to 11.77) were independent risk factors for sudden death.

CONCLUSIONS

Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death. (ClinicalTrials.gov number, NCT00622453.)

Authors+Show Affiliations

Department of Medicine, Krannert Institute of Cardiology, Indiana University, Indianapolis 46202, USA. wgroh@iupui.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

18565861

Citation

Groh, William J., et al. "Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1." The New England Journal of Medicine, vol. 358, no. 25, 2008, pp. 2688-97.
Groh WJ, Groh MR, Saha C, et al. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med. 2008;358(25):2688-97.
Groh, W. J., Groh, M. R., Saha, C., Kincaid, J. C., Simmons, Z., Ciafaloni, E., Pourmand, R., Otten, R. F., Bhakta, D., Nair, G. V., Marashdeh, M. M., Zipes, D. P., & Pascuzzi, R. M. (2008). Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. The New England Journal of Medicine, 358(25), 2688-97. https://doi.org/10.1056/NEJMoa062800
Groh WJ, et al. Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1. N Engl J Med. 2008 Jun 19;358(25):2688-97. PubMed PMID: 18565861.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. AU - Groh,William J, AU - Groh,Miriam R, AU - Saha,Chandan, AU - Kincaid,John C, AU - Simmons,Zachary, AU - Ciafaloni,Emma, AU - Pourmand,Rahman, AU - Otten,Richard F, AU - Bhakta,Deepak, AU - Nair,Girish V, AU - Marashdeh,Mohammad M, AU - Zipes,Douglas P, AU - Pascuzzi,Robert M, PY - 2008/6/21/pubmed PY - 2008/6/27/medline PY - 2008/6/21/entrez SP - 2688 EP - 97 JF - The New England journal of medicine JO - N. Engl. J. Med. VL - 358 IS - 25 N2 - BACKGROUND: Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise. METHODS: We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or third-degree atrioventricular block. RESULTS: Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter-defibrillator during the follow-up period. During a mean follow-up period of 5.7 years, 81 patients died; there were 27 sudden deaths, 32 deaths from progressive neuromuscular respiratory failure, 5 nonsudden deaths from cardiac causes, and 17 deaths from other causes. Among the 17 patients who died suddenly in whom postcollapse rhythm was evaluated, a ventricular tachyarrhythmia was observed in 9. A severe ECG abnormality (relative risk, 3.30; 95% confidence interval [CI], 1.24 to 8.78) and a clinical diagnosis of atrial tachyarrhythmia (relative risk, 5.18; 95% CI, 2.28 to 11.77) were independent risk factors for sudden death. CONCLUSIONS: Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death. (ClinicalTrials.gov number, NCT00622453.) SN - 1533-4406 UR - https://www.unboundmedicine.com/medline/citation/18565861/Electrocardiographic_abnormalities_and_sudden_death_in_myotonic_dystrophy_type_1_ L2 - http://www.nejm.org/doi/full/10.1056/NEJMoa062800?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -