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

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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.; Svendsen, Jesper Hastrup; Edvardsen, Thor; Haugaa, Kristina H.

In: International Journal of Cardiology, Vol. 317, 15.10.2020, p. 152-158.

Research output: Contribution to journalArticle

Harvard

Stokke, MK, Castrini, AI, Aneq, MÅ, Jensen, HK, Madsen, T, Hansen, J, Bundgaard, H, Gilljam, T, Platonov, PG, Svendsen, JH, Edvardsen, T & Haugaa, KH 2020, 'Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients', International Journal of Cardiology, vol. 317, pp. 152-158. https://doi.org/10.1016/j.ijcard.2020.05.095

APA

Stokke, M. K., Castrini, A. I., Aneq, M. Å., Jensen, H. K., Madsen, T., Hansen, J., Bundgaard, H., Gilljam, T., Platonov, P. G., Svendsen, J. H., Edvardsen, T., & Haugaa, K. H. (2020). Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients. International Journal of Cardiology, 317, 152-158. https://doi.org/10.1016/j.ijcard.2020.05.095

CBE

Stokke MK, Castrini AI, Aneq MÅ, Jensen HK, Madsen T, Hansen J, Bundgaard H, Gilljam T, Platonov PG, Svendsen JH, Edvardsen T, Haugaa KH. 2020. Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients. International Journal of Cardiology. 317:152-158. https://doi.org/10.1016/j.ijcard.2020.05.095

MLA

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Author

Stokke, Mathis K. ; Castrini, Anna I. ; Aneq, Meriam Åström ; Jensen, Henrik Kjærulf ; Madsen, Trine ; Hansen, Jim ; Bundgaard, Henning ; Gilljam, Thomas ; Platonov, Pyotr G. ; Svendsen, Jesper Hastrup ; Edvardsen, Thor ; Haugaa, Kristina H. / Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients. In: International Journal of Cardiology. 2020 ; Vol. 317. pp. 152-158.

RIS

TY - JOUR

T1 - Absence of ECG Task Force Criteria does not rule out structural changes in genotype positive ARVC patients

AU - Stokke, Mathis K.

AU - Castrini, Anna I.

AU - Aneq, Meriam Åström

AU - Jensen, Henrik Kjærulf

AU - Madsen, Trine

AU - Hansen, Jim

AU - Bundgaard, Henning

AU - Gilljam, Thomas

AU - Platonov, Pyotr G.

AU - Svendsen, Jesper Hastrup

AU - Edvardsen, Thor

AU - Haugaa, Kristina H.

PY - 2020/10/15

Y1 - 2020/10/15

N2 - 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.

AB - 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.

KW - Arrhythmogenic Right Ventricular Cardiomyopathy

KW - Cardiac magnetic resonance imaging

KW - Diagnostics

KW - ECG

KW - Echocardiography

KW - Pathophysiology

U2 - 10.1016/j.ijcard.2020.05.095

DO - 10.1016/j.ijcard.2020.05.095

M3 - Article

C2 - 32504717

AN - SCOPUS:85086518538

VL - 317

SP - 152

EP - 158

JO - International Journal of Cardiology

JF - International Journal of Cardiology

SN - 0167-5273

ER -