High prevalence of cardiac involvement in patients with myotonic dystrophy type 1: A cross-sectional study

Helle Petri; Nanna Witting; Mads Kristian Ersbøll; Ahmad Sajadieh; Morten Dunø; Susanne Helweg-Larsen; John Vissing; Lars Køber; Henning Bundgaard

doi:10.1016/j.ijcard.2014.03.088

High prevalence of cardiac involvement in patients with myotonic dystrophy type 1: A cross-sectional study

Helle Petri, Nanna Witting, Mads Kristian Ersbøll, Ahmad Sajadieh, Morten Dunø, Susanne Helweg-Larsen, John Vissing, Lars Køber, Henning Bundgaard

29 Citations (Scopus)

Abstract

BACKGROUND: Patients with myotonic dystrophy type 1 (DM1) have a three-fold higher risk of sudden cardiac death (SCD) than age-matched healthy controls. Despite numerous attempts to define the cardiac phenotype and natural history, existing literature suffers from low power, selection-bias and lack of controls. Thus, the optimal strategy for assessing cardiac involvement in DM1 is unclear.

METHOD: In this large single-centre study, we evaluated 129 unselected DM1 patients (49.6% men), mean (SD) age 44 (14.7) years with family history, physical examination, electrocardiogram (ECG), echocardiography, Holter-monitoring and muscle strength testing.

RESULTS: Cardiac involvement was found in 71 patients (55%) and included: 1) Conduction abnormalities: atrio-ventricular block grade I (AVB grade I) (23.6%), AVB grade II (5.6%), right/left bundle branch block (5.5/3.2%) and prolonged QTc (7.2%); 2) arrhythmias: atrial fibrillation/flutter (4.1%), other supraventricular tachyarrhythmia (7.3%) and non-sustained ventricular tachycardia (4.1%); and 3) structural abnormalities: left ventricular systolic dysfunction (20.6%) and reduced global longitudinal strain (21.7%). A normal ECG was not significantly associated with normal findings on Holter-monitoring or echocardiography. Patients with abnormal cardiac findings had weaker muscle strength than those with normal cardiac findings: ankle dorsal flexion (median (range) 4.5 (0-5) vs. 5.0 (2.5-5), p=0.004) and handgrip (median 4.0 (0-5) vs. 4.50 (2-5), p=0.02).

CONCLUSION: The cardiac phenotype of DM1 includes a high prevalence of conduction disorders, arrhythmias and risk factors of SCD. Systematic cardiac screening with ECG, Holter-monitoring and echocardiography is needed in order to make a proper characterization of cardiac involvement in DM1.

Original language	English
Journal	International Journal of Cardiology
Volume	174
Issue number	1
Pages (from-to)	31-36
Number of pages	6
ISSN	0167-5273
DOIs	https://doi.org/10.1016/j.ijcard.2014.03.088
Publication status	Published - 1 Jun 2014

Keywords

Adult
Cross-Sectional Studies
Female
Heart Diseases
Humans
Male
Myotonic Dystrophy
Prevalence

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10.1016/j.ijcard.2014.03.088

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@article{d39356e5be174f24aa48d08d5ca4155c,

title = "High prevalence of cardiac involvement in patients with myotonic dystrophy type 1: A cross-sectional study",

abstract = "BACKGROUND: Patients with myotonic dystrophy type 1 (DM1) have a three-fold higher risk of sudden cardiac death (SCD) than age-matched healthy controls. Despite numerous attempts to define the cardiac phenotype and natural history, existing literature suffers from low power, selection-bias and lack of controls. Thus, the optimal strategy for assessing cardiac involvement in DM1 is unclear.METHOD: In this large single-centre study, we evaluated 129 unselected DM1 patients (49.6% men), mean (SD) age 44 (14.7) years with family history, physical examination, electrocardiogram (ECG), echocardiography, Holter-monitoring and muscle strength testing.RESULTS: Cardiac involvement was found in 71 patients (55%) and included: 1) Conduction abnormalities: atrio-ventricular block grade I (AVB grade I) (23.6%), AVB grade II (5.6%), right/left bundle branch block (5.5/3.2%) and prolonged QTc (7.2%); 2) arrhythmias: atrial fibrillation/flutter (4.1%), other supraventricular tachyarrhythmia (7.3%) and non-sustained ventricular tachycardia (4.1%); and 3) structural abnormalities: left ventricular systolic dysfunction (20.6%) and reduced global longitudinal strain (21.7%). A normal ECG was not significantly associated with normal findings on Holter-monitoring or echocardiography. Patients with abnormal cardiac findings had weaker muscle strength than those with normal cardiac findings: ankle dorsal flexion (median (range) 4.5 (0-5) vs. 5.0 (2.5-5), p=0.004) and handgrip (median 4.0 (0-5) vs. 4.50 (2-5), p=0.02).CONCLUSION: The cardiac phenotype of DM1 includes a high prevalence of conduction disorders, arrhythmias and risk factors of SCD. Systematic cardiac screening with ECG, Holter-monitoring and echocardiography is needed in order to make a proper characterization of cardiac involvement in DM1.",

keywords = "Adult, Cross-Sectional Studies, Female, Heart Diseases, Humans, Male, Myotonic Dystrophy, Prevalence",

author = "Helle Petri and Nanna Witting and Ersb{\o}ll, {Mads Kristian} and Ahmad Sajadieh and Morten Dun{\o} and Susanne Helweg-Larsen and John Vissing and Lars K{\o}ber and Henning Bundgaard",

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doi = "10.1016/j.ijcard.2014.03.088",

language = "English",

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T1 - High prevalence of cardiac involvement in patients with myotonic dystrophy type 1

T2 - A cross-sectional study

AU - Petri, Helle

AU - Witting, Nanna

AU - Ersbøll, Mads Kristian

AU - Sajadieh, Ahmad

AU - Dunø, Morten

AU - Helweg-Larsen, Susanne

AU - Vissing, John

AU - Køber, Lars

AU - Bundgaard, Henning

PY - 2014/6/1

Y1 - 2014/6/1

N2 - BACKGROUND: Patients with myotonic dystrophy type 1 (DM1) have a three-fold higher risk of sudden cardiac death (SCD) than age-matched healthy controls. Despite numerous attempts to define the cardiac phenotype and natural history, existing literature suffers from low power, selection-bias and lack of controls. Thus, the optimal strategy for assessing cardiac involvement in DM1 is unclear.METHOD: In this large single-centre study, we evaluated 129 unselected DM1 patients (49.6% men), mean (SD) age 44 (14.7) years with family history, physical examination, electrocardiogram (ECG), echocardiography, Holter-monitoring and muscle strength testing.RESULTS: Cardiac involvement was found in 71 patients (55%) and included: 1) Conduction abnormalities: atrio-ventricular block grade I (AVB grade I) (23.6%), AVB grade II (5.6%), right/left bundle branch block (5.5/3.2%) and prolonged QTc (7.2%); 2) arrhythmias: atrial fibrillation/flutter (4.1%), other supraventricular tachyarrhythmia (7.3%) and non-sustained ventricular tachycardia (4.1%); and 3) structural abnormalities: left ventricular systolic dysfunction (20.6%) and reduced global longitudinal strain (21.7%). A normal ECG was not significantly associated with normal findings on Holter-monitoring or echocardiography. Patients with abnormal cardiac findings had weaker muscle strength than those with normal cardiac findings: ankle dorsal flexion (median (range) 4.5 (0-5) vs. 5.0 (2.5-5), p=0.004) and handgrip (median 4.0 (0-5) vs. 4.50 (2-5), p=0.02).CONCLUSION: The cardiac phenotype of DM1 includes a high prevalence of conduction disorders, arrhythmias and risk factors of SCD. Systematic cardiac screening with ECG, Holter-monitoring and echocardiography is needed in order to make a proper characterization of cardiac involvement in DM1.

AB - BACKGROUND: Patients with myotonic dystrophy type 1 (DM1) have a three-fold higher risk of sudden cardiac death (SCD) than age-matched healthy controls. Despite numerous attempts to define the cardiac phenotype and natural history, existing literature suffers from low power, selection-bias and lack of controls. Thus, the optimal strategy for assessing cardiac involvement in DM1 is unclear.METHOD: In this large single-centre study, we evaluated 129 unselected DM1 patients (49.6% men), mean (SD) age 44 (14.7) years with family history, physical examination, electrocardiogram (ECG), echocardiography, Holter-monitoring and muscle strength testing.RESULTS: Cardiac involvement was found in 71 patients (55%) and included: 1) Conduction abnormalities: atrio-ventricular block grade I (AVB grade I) (23.6%), AVB grade II (5.6%), right/left bundle branch block (5.5/3.2%) and prolonged QTc (7.2%); 2) arrhythmias: atrial fibrillation/flutter (4.1%), other supraventricular tachyarrhythmia (7.3%) and non-sustained ventricular tachycardia (4.1%); and 3) structural abnormalities: left ventricular systolic dysfunction (20.6%) and reduced global longitudinal strain (21.7%). A normal ECG was not significantly associated with normal findings on Holter-monitoring or echocardiography. Patients with abnormal cardiac findings had weaker muscle strength than those with normal cardiac findings: ankle dorsal flexion (median (range) 4.5 (0-5) vs. 5.0 (2.5-5), p=0.004) and handgrip (median 4.0 (0-5) vs. 4.50 (2-5), p=0.02).CONCLUSION: The cardiac phenotype of DM1 includes a high prevalence of conduction disorders, arrhythmias and risk factors of SCD. Systematic cardiac screening with ECG, Holter-monitoring and echocardiography is needed in order to make a proper characterization of cardiac involvement in DM1.

KW - Adult

KW - Cross-Sectional Studies

KW - Female

KW - Heart Diseases

KW - Humans

KW - Male

KW - Myotonic Dystrophy

KW - Prevalence

U2 - 10.1016/j.ijcard.2014.03.088

DO - 10.1016/j.ijcard.2014.03.088

M3 - Journal article

C2 - 24704412

SN - 0167-5273

VL - 174

SP - 31

EP - 36

JO - International Journal of Cardiology

JF - International Journal of Cardiology

IS - 1

ER -

High prevalence of cardiac involvement in patients with myotonic dystrophy type 1: A cross-sectional study

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Keywords

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