Severe Cardiac Arrhythmias in Young Patients with Myotonic Dystrophy Type 1

Overview

Journal Neurology

Specialty Neurology

Date 2004 Nov 24

PMID 15557517

Citations 20

Authors

G Bassez

A Lazarus

I Desguerre

J Varin

P Laforet

H M Becane

C Meune

M C Arne-Bes

Z Ounnoughene

H Radvanyi

B Eymard

D Duboc

Affiliations

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Abstract

Cardiac tachyarrhythmias have rarely been studied in young patients with myotonic dystrophy type 1 (DM1). The authors observed major cardiac rhythm disturbances in 11 patients aged 10 to 18 years. Tachyarrhythmic events were more frequent than impulse conduction disorders. Wide variations in CTG expansion were observed among the population. Since physical exercise was a prominent arrhythmogenic factor, systematic exercise tests with EKG monitoring may be indicated in young patients with DM1.

Citing Articles

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Cardiac Manifestations of Myotonic Dystrophy in a Pediatric Cohort.

Brunet Garcia L, Hajra A, Field E, Wacher J, Walsh H, Norrish G Front Pediatr. 2022; 10:910660.

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Mice lacking MBNL1 and MBNL2 exhibit sudden cardiac death and molecular signatures recapitulating myotonic dystrophy.

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Cardiac Pathology in Myotonic Dystrophy Type 1.

Mahadevan M, Yadava R, Mandal M Int J Mol Sci. 2021; 22(21).

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Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy.

McNally E, Mann D, Pinto Y, Bhakta D, Tomaselli G, Nazarian S J Am Heart Assoc. 2020; 9(4):e014006.

PMID: 32067592 PMC: 7070199. DOI: 10.1161/JAHA.119.014006.