Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1

William J. Groh, Miriam R. Groh, Chandan Saha, John C. Kincaid, Zachary Simmons, Emma Ciafaloni, Rahman Pourmand, Richard F. Otten, Deepak Bhakta, Girish V. Nair, Mohammad M. Marashdeh, Douglas P. Zipes, Robert M. Pascuzzi

Research output: Contribution to journalArticle

290 Citations (Scopus)

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.)

Original languageEnglish (US)
Pages (from-to)2688-2697
Number of pages10
JournalNew England Journal of Medicine
Volume358
Issue number25
DOIs
StatePublished - Jun 19 2008

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Myotonic Dystrophy
Sudden Death
Electrocardiography
Tachycardia
Confidence Intervals
Neuromuscular Diseases
Implantable Defibrillators
Atrioventricular Block
Left Ventricular Dysfunction
Respiratory Insufficiency
Cardiac Arrhythmias
Cause of Death
Skeletal Muscle
Heart Failure

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Groh, W. J., Groh, M. R., Saha, C., Kincaid, J. C., Simmons, Z., Ciafaloni, E., ... Pascuzzi, R. M. (2008). Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. New England Journal of Medicine, 358(25), 2688-2697. https://doi.org/10.1056/NEJMoa062800
Groh, William J. ; Groh, Miriam R. ; Saha, Chandan ; Kincaid, John C. ; Simmons, Zachary ; Ciafaloni, Emma ; Pourmand, Rahman ; Otten, Richard F. ; Bhakta, Deepak ; Nair, Girish V. ; Marashdeh, Mohammad M. ; Zipes, Douglas P. ; Pascuzzi, Robert M. / Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. In: New England Journal of Medicine. 2008 ; Vol. 358, No. 25. pp. 2688-2697.
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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.)",
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Groh, WJ, Groh, MR, Saha, C, Kincaid, JC, Simmons, Z, Ciafaloni, E, Pourmand, R, Otten, RF, Bhakta, D, Nair, GV, Marashdeh, MM, Zipes, DP & Pascuzzi, RM 2008, 'Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1', New England Journal of Medicine, vol. 358, no. 25, pp. 2688-2697. https://doi.org/10.1056/NEJMoa062800

Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. / Groh, William J.; Groh, Miriam R.; Saha, Chandan; Kincaid, John C.; Simmons, Zachary; Ciafaloni, Emma; Pourmand, Rahman; Otten, Richard F.; Bhakta, Deepak; Nair, Girish V.; Marashdeh, Mohammad M.; Zipes, Douglas P.; Pascuzzi, Robert M.

In: New England Journal of Medicine, Vol. 358, No. 25, 19.06.2008, p. 2688-2697.

Research output: Contribution to journalArticle

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 - 2008/6/19

Y1 - 2008/6/19

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.)

AB - 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.)

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