A Normal Electrocardiogram Does Not Exclude Infra-Hisian Conduction Disease in Patients With Myotonic Dystrophy Type 1.
electrocardiogram
electrophysiology study
myotonic dystrophy
permanent pacemaker
sudden death
Journal
JACC. Clinical electrophysiology
ISSN: 2405-5018
Titre abrégé: JACC Clin Electrophysiol
Pays: United States
ID NLM: 101656995
Informations de publication
Date de publication:
08 2021
08 2021
Historique:
received:
23
09
2020
revised:
11
01
2021
accepted:
12
01
2021
pubmed:
5
4
2021
medline:
26
10
2021
entrez:
4
4
2021
Statut:
ppublish
Résumé
This study aimed to identify electrocardiographic (ECG) predictors of a prolonged His-ventricular (HV) interval in patients with type 1 myotonic dystrophy (DM1). Patients with DM1 have an increased risk of sudden cardiac death. The presence of His-Purkinje system disease/prolonged HV interval (≥70 ms) is associated with a higher risk of potentially life-threatening bradyarrhythmic events. Electrophysiology studies (EPSs) were performed in all DM1 patients referred to 2 tertiary centers for routine cardiac assessment. In a subgroup of patients, the EPS was repeated at varying intervals. A total of 154 patients (mean age: 43.7 ± 13.3; 58.1% male) underwent 202 diagnostic EPSs. HV ≥70 ms was found on 58 EPSs (28.7%); 9 of 59 patients (15.2%) with PR <200 ms and QRS interval <110 ms on baseline ECG had an HV ≥70 ms on EPS. Among those with PR ≥200 ms and/or QRS interval ≥100 ms, only 33.9% had an HV ≥70 ms on EPS. There were 38 patients who underwent repeated EPS, in which 28.8% demonstrated a prolongation of the HV interval overall compared with baseline. QRS duration demonstrated the most powerful discriminative capacity for HV ≥70 ms (area under the receiver operating characteristic curve: 0.76; 95% confidence interval [CI]: 0.68 to 0.84; p < 0.001). On multivariate analysis, QRS interval ≥112 ms had the highest predictive value for HV ≥70 ms (odds ratio: 7.94; 95% CI: 3.85 to 16.37. ECG parameters have a poor predictive value for infra-Hisian conduction block in DM1 patients. QRS and PR intervals are normal in up to 15.2% of DM1 patients with prolonged HV, and 66.1% of those with PR ≥200 ms and/or QRS ≥100 ms do not have advanced His-Purkinje conduction system disease on EPS. Electrophysiology testing should be a mandatory part of screening for all patients to guide prophylactic pacemaker implantation.
Sections du résumé
OBJECTIVES
This study aimed to identify electrocardiographic (ECG) predictors of a prolonged His-ventricular (HV) interval in patients with type 1 myotonic dystrophy (DM1).
BACKGROUND
Patients with DM1 have an increased risk of sudden cardiac death. The presence of His-Purkinje system disease/prolonged HV interval (≥70 ms) is associated with a higher risk of potentially life-threatening bradyarrhythmic events.
METHODS
Electrophysiology studies (EPSs) were performed in all DM1 patients referred to 2 tertiary centers for routine cardiac assessment. In a subgroup of patients, the EPS was repeated at varying intervals.
RESULTS
A total of 154 patients (mean age: 43.7 ± 13.3; 58.1% male) underwent 202 diagnostic EPSs. HV ≥70 ms was found on 58 EPSs (28.7%); 9 of 59 patients (15.2%) with PR <200 ms and QRS interval <110 ms on baseline ECG had an HV ≥70 ms on EPS. Among those with PR ≥200 ms and/or QRS interval ≥100 ms, only 33.9% had an HV ≥70 ms on EPS. There were 38 patients who underwent repeated EPS, in which 28.8% demonstrated a prolongation of the HV interval overall compared with baseline. QRS duration demonstrated the most powerful discriminative capacity for HV ≥70 ms (area under the receiver operating characteristic curve: 0.76; 95% confidence interval [CI]: 0.68 to 0.84; p < 0.001). On multivariate analysis, QRS interval ≥112 ms had the highest predictive value for HV ≥70 ms (odds ratio: 7.94; 95% CI: 3.85 to 16.37.
CONCLUSIONS
ECG parameters have a poor predictive value for infra-Hisian conduction block in DM1 patients. QRS and PR intervals are normal in up to 15.2% of DM1 patients with prolonged HV, and 66.1% of those with PR ≥200 ms and/or QRS ≥100 ms do not have advanced His-Purkinje conduction system disease on EPS. Electrophysiology testing should be a mandatory part of screening for all patients to guide prophylactic pacemaker implantation.
Identifiants
pubmed: 33812832
pii: S2405-500X(21)00108-0
doi: 10.1016/j.jacep.2021.01.017
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1038-1048Commentaires et corrections
Type : CommentIn
Type : CommentIn
Type : CommentIn
Informations de copyright
Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.