Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients

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  • Mathis K Stokke, University of Oslo, Rikshospitalet Oslo
  • ,
  • Anna I Castrini, Rikshospitalet Oslo
  • ,
  • Meriam Åström Aneq, Linköping University
  • ,
  • Henrik Kjærulf Jensen
  • Trine Madsen
  • ,
  • Jim Hansen, University of Copenhagen
  • ,
  • Henning Bundgaard, University of Copenhagen, Rigshospitalet
  • ,
  • Thomas Gilljam, University of Gothenburg
  • ,
  • Pyotr G Platonov, Skåne University Hospital, Lund
  • ,
  • Jesper Hastrup Svendsen, University of Copenhagen, Rigshospitalet
  • ,
  • Thor Edvardsen, University of Oslo, Rikshospitalet Oslo
  • ,
  • Kristina H Haugaa, University of Oslo, Rikshospitalet Oslo

AIMS: In Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), electrophysiological pathology has been claimed to precede morphological and functional pathology. Accordingly, an ECG without ARVC markers should be rare in ARVC patients with pathology identified by cardiac imaging. We quantified the prevalence of ARVC patients with evidence of structural disease, yet without ECG Task Force Criteria (TFC).

METHODS AND RESULTS: We included 182 probands and family members with ARVC-associated mutations (40 ± 17 years, 50% women, 73% PKP2 mutations) from the Nordic ARVC Registry in a cross-sectional analysis. For echocardiography and cardiac MR (CMR), we differentiated between "abnormalities" and TFC. "Abnormalities" were defined as RV functional or structural measures outside TFC reference values, without combinations required to fulfill TFC. ECG TFC were used as defined, as these are not composite parameters. We found that only 4% of patients with ARVC fulfilled echocardiographic TFC without any ECG TFC. However, importantly, 38% of patients had imaging abnormalities without any ECG TFC. These results were supported by CMR data from a subset of 51 patients: 16% fulfilled CMR TFC without fulfilling ECG TFC, while 24% had CMR abnormalities without any ECG TFC. In a multivariate analysis, echocardiographic TFC were associated with arrhythmic events.

CONCLUSION: More than one third of ARVC genotype positive patients had subtle imaging abnormalities without fulfilling ECG TFC. Although most patients will have both imaging and ECG abnormalities, structural abnormalities in ARVC genotype positive patients cannot be ruled out by the absence of ECG TFC.

Original languageEnglish
JournalInternational Journal of Cardiology
Volume317
Pages (from-to)152-158
Number of pages7
ISSN0167-5273
DOIs
Publication statusPublished - Oct 2020

Bibliographical note

Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.

    Research areas

  • Arrhythmogenic Right Ventricular Cardiomyopathy, Cardiac magnetic resonance imaging, Diagnostics, ECG, Echocardiography, Pathophysiology

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