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Repolarisation abnormalities unmasked with a 252-lead BSM system in patients with ARVC and healthy Gene Carriers
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Cardiology-Arrhythmia.ORCID iD: 0000-0002-0827-5629
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Cardiology-Arrhythmia.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Cardiology-Arrhythmia.ORCID iD: 0000-0003-2806-3903
2022 (English)In: Pacing and Clinical Electrophysiology, ISSN 0147-8389, E-ISSN 1540-8159, Vol. 45, no 4, p. 509-518Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) at an early stage can be challenging even after ECG recording and a combination of several imaging techniques. The purpose of this study was to explore if a Body Surface Mapping (BSM) system with 252-leads could identify repolarization abnormalities and thereby diagnose early stages of ARVC.

METHODS: ARVC patients, gene carriers without signs of ARVC and controls underwent a 12 lead resting ECG, signal-averaged ECG, echocardiography, 24-hours Holter monitoring and BSM with electrocardiographic imaging (ECGI). All 252-leads, divided into four quadrants of the vest, were analyzed regarding concordances between T wave polarity and QRS main vector.

RESULTS: Of 40 patients included there were 12 ARVC patients, 20 gene carriers and 8 controls. The ARVC patients had two different repolarization patterns, one with more pronounced negative T waves at the lower left panel and another with mixed changes that clearly differed from the controls, all of whom had a normal 12 lead ECGs and consistent repolarization patterns on their BSM recordings. The patterns observed in ARVC patients were also present in 5/20 (25%) gene carriers, three of whom had normal resting ECG. A novel repolarization index successfully detected all ARVC patients and 88% of gene carriers with pathologic repolarization pattern.

CONCLUSIONS: The finding that abnormal repolarization patterns could be unmasked by BSM in 25% of healthy gene carriers, suggests that it may potentially be a useful tool for identifying early manifestations of ARVC. Further and larger studies are warranted to assess its diagnostic accuracy.

Place, publisher, year, edition, pages
John Wiley & Sons, 2022. Vol. 45, no 4, p. 509-518
Keywords [en]
Arrhythmogenic, Body Surface Mapping, Cardiomyopathy, Right Ventricular, repolarization
National Category
Medical and Health Sciences
Research subject
Cardiology
Identifiers
URN: urn:nbn:se:uu:diva-467328DOI: 10.1111/pace.14456ISI: 000768411000001PubMedID: 35077593OAI: oai:DiVA.org:uu-467328DiVA, id: diva2:1636512
Funder
Erik, Karin och Gösta Selanders FoundationMedtronic, SwedenSwedish Heart Lung Foundation, 20150751Available from: 2022-02-10 Created: 2022-02-10 Last updated: 2022-09-05Bibliographically approved
In thesis
1. Novel approaches using electrocardiographic imaging for early detection of ARVC in patients and relatives and symptoms preceding sudden death
Open this publication in new window or tab >>Novel approaches using electrocardiographic imaging for early detection of ARVC in patients and relatives and symptoms preceding sudden death
2022 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited disease of the myocardium, predominantly affecting the right ventricle (RV). Arrhythmias are common among patients with the disease and Sudden Cardiac Death (SCD) can occur even in early stages. 

The overall purpose of this thesis was to investigate the effectiveness of new diagnostic methods in detecting early abnormalities in genetically predisposed individuals and to emphasize the importance of early diagnosis. 

The analysis of body surface mapping (BSM) signals recorded using a 252-lead vest revealed abnormal repolarisation patterns in all ARVC patients, but also in 25% of family members who were carriers of the family pathogenic variant (M-carriers). The abnormal repolarization patterns preceded repolarization abnormalities on 12-leads electrocardiogram (ECG). Depolarization abnormalities were also detected by the analysis of body surface signals. The QRS dispersion calculated by the body surface signals was significantly higher among ARVC patients compared with controls. 20% of M-carriers presented also with a slightly elevated QRS dispersion. ECG based QRS dispersion could not adequately differentiate ARVC patients from controls. Thus, the higher resolution of the BSM system permitted the detection of repolarization and depolarization abnormalities even in early stages of the disease.

The analysis of reconstructed epicardial signals using Electrocardiographic Imaging (ECGI) revealed terminal ventricular epicardial activation (the last 20msecs) located only in parts of RV, as opposed to controls, where the right ventricular outflow tract (RVOT) and cardiac base (both right and left ventricle) were activated last. The total ventricular activation time and the RV activation time were both longer in ARVC patients, whereas the area activated during the last 20 msecs was smaller. Similar pattern with delayed conduction in limited areas of the RV were also observed in 50% of the M-carriers. This subgroup presented also smaller area of terminal ventricular activation and longer RV activation time, but the total ventricular activation was normal. 

Through nationwide registries, the first SCD cohort due to ARVC in Sweden was described. Cardiac related symptoms were common (68%) prior to death and 36% of cases had sought medical care the last six months prior to death. A family history of SCD was present in 45% of the cases.  The careful clinical evaluation of young individuals seeking with cardiac related symptoms and the evaluation of both medical and family history is crucial.

In conclusion, new technologies, using multiple electrodes for the recording of body surface signals and the reconstruction of the epicardial signals have shown promising results in detecting early repolarization and depolarization abnormalities and could facilitate the early diagnosis in M-carriers.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2022. p. 106
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 1847
Keywords
Arrhythmogenic Right Ventricular Cardiomyopathy, diagnosis, gene carriers, body surface mapping, electrocardiographic imaging, sudden cardiac death
National Category
Cardiac and Cardiovascular Systems
Research subject
Cardiology; Cardiology
Identifiers
urn:nbn:se:uu:diva-472816 (URN)978-91-513-1524-9 (ISBN)
Public defence
2022-06-14, Enghoffsalen, Akademiska Sjukhuset, ing 50, Uppsala, 13:00 (English)
Opponent
Supervisors
Available from: 2022-05-23 Created: 2022-04-24 Last updated: 2022-06-15

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Kommata, VarvaraSciaraffia, ElenaBlomström-Lundqvist, Carina

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