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A Normal Electrocardiogram Does Not Exclude Infra-Hisian Conduction Disease in Patients With Myotonic Dystrophy Type 1

Creta, A; Providencia, R; Gossios, T; Elliott, PM; Turner, C; Savvatis, K; Segal, OR; (2021) A Normal Electrocardiogram Does Not Exclude Infra-Hisian Conduction Disease in Patients With Myotonic Dystrophy Type 1. JACC: Clinical Electrophysiology , 7 (8) pp. 1038-1048. 10.1016/j.jacep.2021.01.017. Green open access

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Abstract

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.

Type: Article
Title: A Normal Electrocardiogram Does Not Exclude Infra-Hisian Conduction Disease in Patients With Myotonic Dystrophy Type 1
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jacep.2021.01.017
Publisher version: http://dx.doi.org/10.1016/j.jacep.2021.01.017
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: electrocardiogram, electrophysiology study, permanent pacemaker, myotonic dystrophy, sudden death, MORTALITY, ARRHYTHMIAS, PREDICTORS, PACEMAKER, TIME
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Health Informatics
URI: https://discovery.ucl.ac.uk/id/eprint/10139969
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