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Epicardial conduction abnormalities in patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) and mutation positive healthy family members – a study using electrocardiographic imaging
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. Örebro Univ, Fac Med & Hlth, Sch Med Sci, Dept Cardiol, Örebro, Sweden..ORCID iD: 0000-0003-2806-3903
2023 (English)In: PLOS ONE, E-ISSN 1932-6203, Vol. 18, no 1, article id e0280111Article in journal (Refereed) Published
Abstract [en]

Background: The diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) in early stages is challenging. The aim was therefore to study whether electrocardiographic imaging (ECGI) can detect epicardial depolarization changes in ARVC patients and healthy mutation-carriers (M-carriers).

Method: Twelve ARVC patients, 20 M-carriers and 8 controls underwent 12-lead ECG, signal-averaged ECG, 2-dimensional echocardiography, 24-hours Holter monitoring and ECGI (body surface mapping and computer tomography with offline analysis of reconstructed epicardial signals). Total and Right Ventricular Activation Time (tVAT and RVAT respectively), area of Ventricular Activation during the terminal 20 milliseconds (aVAte20) and the activation patterns were compared between groups.

Results: In ARVC patients the locations of aVAte20 were scattered or limited to smaller RV parts versus in controls, in whom aVAte20 was confined to RVOT and LV base (+/- RV base). ARVC patients had smaller aVAte20 (35cm2 vs 87cm2, p<0.05), longer tVAT (99msec vs 58msec, p<0.05) and longer RVAT (66msec vs 43msec, p<0.05) versus controls. In 50% of M-carriers, the locations of aVAte20 were also eccentric. This sub-group presented smaller aVAte20 (53cm2 vs 87cm2, p= 0.009), longer RVAT (55msec vs 48msec, p=0.043), but similar tVAT (65msec vs 60msec, p=0.529) compared with the M-carriers with normal activation pattern.

Conclusions: The observation of localized delayed epicardial conduction in the RV in M-carriers suggests an early stage of ARVC and may be a useful diagnostic marker enhancing an early detection of the disease.

Place, publisher, year, edition, pages
Public Library of Science (PLoS), 2023. Vol. 18, no 1, article id e0280111
Keywords [en]
Arrhythmogenic Right Ventricular Cardiomyopathy, diagnosis, gene carriers, electrocardiographic imaging
National Category
Cardiac and Cardiovascular Systems
Research subject
Cardiology
Identifiers
URN: urn:nbn:se:uu:diva-473210DOI: 10.1371/journal.pone.0280111ISI: 000945431500001PubMedID: 36603020OAI: oai:DiVA.org:uu-473210DiVA, id: diva2:1653688
Available from: 2022-04-22 Created: 2022-04-22 Last updated: 2023-04-03Bibliographically 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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