Cardiac Manifestations of Myotonic Dystrophy in a Pediatric Cohort.
cardiac conduction disease
congenital myotonic dystrophy
electrocardiographic abnormalities
myotonic dystrophy (DM1)
neuromuscular disorder
pediatric population
Journal
Frontiers in pediatrics
ISSN: 2296-2360
Titre abrégé: Front Pediatr
Pays: Switzerland
ID NLM: 101615492
Informations de publication
Date de publication:
2022
2022
Historique:
received:
01
04
2022
accepted:
19
05
2022
entrez:
27
6
2022
pubmed:
28
6
2022
medline:
28
6
2022
Statut:
epublish
Résumé
Myotonic dystrophy type 1 (DM1) is the most prevalent inherited neuromuscular dystrophy in adults. It is a multisystem disease with cardiac manifestations. Whilst these are well-defined in adults, there are scarce published data in the pediatric population. This study aimed to investigate the yield and progression of cardiac disease in pediatric DM1 patients, focusing on congenital DM1 (cDM1). A retrospective observational study of all pediatric DM1 patients referred to our center (December 2000-November 2020) was conducted. Patients were classified into DM1 forms according to age of symptom onset and disease severity. Patients underwent clinical and cardiac evaluation with 12-lead ECG, transthoracic echocardiography and 24-h ECG Holter monitoring. 67 DM1 pediatric patients were included: 56 (83.6%) cDM1 and 11 (16.4%) non-cDM1. Median follow-up time of cDM1 patients was 8.0 [3.25-11.0] years. 49 (87.5%) cDM1 patients had baseline 12-lead ECG and 44 (78.6%) had a follow-up 12-lead-ECG, with a median follow-up time from diagnosis to baseline ECG of 2.8 [1.0-8.5] years and to follow-up ECG of 10.9 [5.7-14.2] years. Overall, 43 (87.8%) presented ECG abnormalities, most commonly in the form of asymptomatic conduction disease ( This study is the first to analyse the prevalence and progression of ECG abnormalities in cDM1 pediatric patients. The high prevalence of abnormal findings, progressive changes and number of potentially associated events (1 pacemaker implantation and 3 unexplained sudden deaths) stresses the importance of systematic and continued cardiac evaluation of these patients.
Identifiants
pubmed: 35757141
doi: 10.3389/fped.2022.910660
pmc: PMC9218560
doi:
Types de publication
Journal Article
Langues
eng
Pagination
910660Subventions
Organisme : Medical Research Council
ID : MR/T024062/1
Pays : United Kingdom
Informations de copyright
Copyright © 2022 Brunet Garcia, Hajra, Field, Wacher, Walsh, Norrish, Manzur, Muntoni, Munot, Robb, Quinlivan, Scoto, Baranello, Sarkozy, Starling, Kaski and Cervi.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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