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

Stokke, Mathis K. ; Castrini, Anna I. ; Aneq, Meriam Åström ; Jensen, Henrik Kjærulf ; Madsen, Trine ; Hansen, Jim ; Bundgaard, Henning ; Gilljam, Thomas ; Platonov, Pyotr G. LU and Svendsen, Jesper Hastrup , et al. (2020) In International Journal of Cardiology
Abstract

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... (More)

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.

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keywords
Arrhythmogenic Right Ventricular Cardiomyopathy, Cardiac magnetic resonance imaging, Diagnostics, ECG, Echocardiography, Pathophysiology
in
International Journal of Cardiology
publisher
Elsevier
external identifiers
  • pmid:32504717
  • scopus:85086518538
ISSN
0167-5273
DOI
10.1016/j.ijcard.2020.05.095
language
English
LU publication?
yes
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1e90c078-d947-4946-a54f-42a8a4fbcbbb
date added to LUP
2020-06-29 14:49:42
date last changed
2020-07-01 05:34:42
@article{1e90c078-d947-4946-a54f-42a8a4fbcbbb,
  abstract     = {<p>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.</p>},
  author       = {Stokke, Mathis K. and Castrini, Anna I. and Aneq, Meriam Åström and Jensen, Henrik Kjærulf and Madsen, Trine and Hansen, Jim and Bundgaard, Henning and Gilljam, Thomas and Platonov, Pyotr G. and Svendsen, Jesper Hastrup and Edvardsen, Thor and Haugaa, Kristina H.},
  issn         = {0167-5273},
  language     = {eng},
  publisher    = {Elsevier},
  series       = {International Journal of Cardiology},
  title        = {Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients},
  url          = {http://dx.doi.org/10.1016/j.ijcard.2020.05.095},
  doi          = {10.1016/j.ijcard.2020.05.095},
  year         = {2020},
}