Increased mortality with left ventricular systolic dysfunction and heart failure in adults with myotonic dystrophy type 1

Deepak Bhakta, Miriam R. Groh, Changyu Shen, Robert Pascuzzi, William Groh

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49 Citations (Scopus)

Abstract

Background: Myotonic dystrophy type 1 (DM1) is a neurologic disorder with known cardiac involvement, including left ventricular systolic dysfunction (LVSD), heart failure (HF), atrioventricular and intraventricular conduction system disease, and sudden death. We studied the prevalence of these conditions and associated findings in a large population with DM1. Methods: History, physical examination, genetic testing, and electrocardiography were performed on 406 patients with DM1, and cardiac imaging was performed on 180 (44.3%) of these patients. Results: Left ventricular systolic dysfunction and clinical HF were found in 34 (18.9%) of 180 and in 23 (5.7%) of 406 of enrolled subjects, respectively, yielding an overall prevalence of LVSD/HF in 41 (10.1%) of 406. Increasing age, male sex, electrocardiographic conduction abnormalities, presence of atrial and ventricular arrhythmias, and implanted devices were all significantly associated with LVSD/HF, whereas cytosine-thiamine-guanine repeat length and neuromuscular severity score were not. The interval ≥240 milliseconds (relative risk 4.1, 95% CI 1.7-9.6, P = .001) and QRS duration ≥120 milliseconds (relative risk 4.2, 95% CI 2.0-8.5, P < .001) were significant predictors of LVSD/HF. The presence of LVSD/HF was also significantly associated with all-cause death (relative risk 3.9, 95% CI 2.3-6.4, P < .001) and cardiac death (relative risk 5.7, 95% CI 2.6-12.4, P < .001). Conclusions: A significant prevalence of LVSD/HF exists in patients with DM1. The presence of LVSD/HF in DM1 is significantly associated with all-cause and cardiac death.

Original languageEnglish
JournalAmerican Heart Journal
Volume160
Issue number6
DOIs
StatePublished - Dec 2010

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Systolic Heart Failure
Myotonic Dystrophy
Left Ventricular Dysfunction
Mortality
Cause of Death
Thiamine
Cytosine
Guanine
Genetic Testing
Sudden Death
Nervous System Diseases
Physical Examination
Cardiac Arrhythmias
Electrocardiography
Heart Failure
History
Equipment and Supplies

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

@article{839157d7c6ff4b2c9a610cb9dfb00467,
title = "Increased mortality with left ventricular systolic dysfunction and heart failure in adults with myotonic dystrophy type 1",
abstract = "Background: Myotonic dystrophy type 1 (DM1) is a neurologic disorder with known cardiac involvement, including left ventricular systolic dysfunction (LVSD), heart failure (HF), atrioventricular and intraventricular conduction system disease, and sudden death. We studied the prevalence of these conditions and associated findings in a large population with DM1. Methods: History, physical examination, genetic testing, and electrocardiography were performed on 406 patients with DM1, and cardiac imaging was performed on 180 (44.3{\%}) of these patients. Results: Left ventricular systolic dysfunction and clinical HF were found in 34 (18.9{\%}) of 180 and in 23 (5.7{\%}) of 406 of enrolled subjects, respectively, yielding an overall prevalence of LVSD/HF in 41 (10.1{\%}) of 406. Increasing age, male sex, electrocardiographic conduction abnormalities, presence of atrial and ventricular arrhythmias, and implanted devices were all significantly associated with LVSD/HF, whereas cytosine-thiamine-guanine repeat length and neuromuscular severity score were not. The interval ≥240 milliseconds (relative risk 4.1, 95{\%} CI 1.7-9.6, P = .001) and QRS duration ≥120 milliseconds (relative risk 4.2, 95{\%} CI 2.0-8.5, P < .001) were significant predictors of LVSD/HF. The presence of LVSD/HF was also significantly associated with all-cause death (relative risk 3.9, 95{\%} CI 2.3-6.4, P < .001) and cardiac death (relative risk 5.7, 95{\%} CI 2.6-12.4, P < .001). Conclusions: A significant prevalence of LVSD/HF exists in patients with DM1. The presence of LVSD/HF in DM1 is significantly associated with all-cause and cardiac death.",
author = "Deepak Bhakta and Groh, {Miriam R.} and Changyu Shen and Robert Pascuzzi and William Groh",
year = "2010",
month = "12",
doi = "10.1016/j.ahj.2010.07.032",
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T1 - Increased mortality with left ventricular systolic dysfunction and heart failure in adults with myotonic dystrophy type 1

AU - Bhakta, Deepak

AU - Groh, Miriam R.

AU - Shen, Changyu

AU - Pascuzzi, Robert

AU - Groh, William

PY - 2010/12

Y1 - 2010/12

N2 - Background: Myotonic dystrophy type 1 (DM1) is a neurologic disorder with known cardiac involvement, including left ventricular systolic dysfunction (LVSD), heart failure (HF), atrioventricular and intraventricular conduction system disease, and sudden death. We studied the prevalence of these conditions and associated findings in a large population with DM1. Methods: History, physical examination, genetic testing, and electrocardiography were performed on 406 patients with DM1, and cardiac imaging was performed on 180 (44.3%) of these patients. Results: Left ventricular systolic dysfunction and clinical HF were found in 34 (18.9%) of 180 and in 23 (5.7%) of 406 of enrolled subjects, respectively, yielding an overall prevalence of LVSD/HF in 41 (10.1%) of 406. Increasing age, male sex, electrocardiographic conduction abnormalities, presence of atrial and ventricular arrhythmias, and implanted devices were all significantly associated with LVSD/HF, whereas cytosine-thiamine-guanine repeat length and neuromuscular severity score were not. The interval ≥240 milliseconds (relative risk 4.1, 95% CI 1.7-9.6, P = .001) and QRS duration ≥120 milliseconds (relative risk 4.2, 95% CI 2.0-8.5, P < .001) were significant predictors of LVSD/HF. The presence of LVSD/HF was also significantly associated with all-cause death (relative risk 3.9, 95% CI 2.3-6.4, P < .001) and cardiac death (relative risk 5.7, 95% CI 2.6-12.4, P < .001). Conclusions: A significant prevalence of LVSD/HF exists in patients with DM1. The presence of LVSD/HF in DM1 is significantly associated with all-cause and cardiac death.

AB - Background: Myotonic dystrophy type 1 (DM1) is a neurologic disorder with known cardiac involvement, including left ventricular systolic dysfunction (LVSD), heart failure (HF), atrioventricular and intraventricular conduction system disease, and sudden death. We studied the prevalence of these conditions and associated findings in a large population with DM1. Methods: History, physical examination, genetic testing, and electrocardiography were performed on 406 patients with DM1, and cardiac imaging was performed on 180 (44.3%) of these patients. Results: Left ventricular systolic dysfunction and clinical HF were found in 34 (18.9%) of 180 and in 23 (5.7%) of 406 of enrolled subjects, respectively, yielding an overall prevalence of LVSD/HF in 41 (10.1%) of 406. Increasing age, male sex, electrocardiographic conduction abnormalities, presence of atrial and ventricular arrhythmias, and implanted devices were all significantly associated with LVSD/HF, whereas cytosine-thiamine-guanine repeat length and neuromuscular severity score were not. The interval ≥240 milliseconds (relative risk 4.1, 95% CI 1.7-9.6, P = .001) and QRS duration ≥120 milliseconds (relative risk 4.2, 95% CI 2.0-8.5, P < .001) were significant predictors of LVSD/HF. The presence of LVSD/HF was also significantly associated with all-cause death (relative risk 3.9, 95% CI 2.3-6.4, P < .001) and cardiac death (relative risk 5.7, 95% CI 2.6-12.4, P < .001). Conclusions: A significant prevalence of LVSD/HF exists in patients with DM1. The presence of LVSD/HF in DM1 is significantly associated with all-cause and cardiac death.

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