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Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria.
J Cardiovasc Electrophysiol. 2008 Aug; 19(8):775-81.JC

Abstract

INTRODUCTION

Desmosomal changes, electrical uncoupling, and surviving myocardial bundles embedded in fibrofatty tissue are hallmarks of activation delay in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Currently, generally accepted task force criteria (TFC) are used for clinical diagnosis. We propose additional criteria based on activation delay and ventricular tachycardia (VT) to improve identification of affected individuals.

METHODS AND RESULTS

Activation delay and VT-related 12-lead electrocardiographic (ECG) criteria were studied, while off drugs, in 42 patients with proven ARVD/C according to TFC, and 27 controls with idiopathic VT from the RV outflow tract. Two of three measured TFC could only be identified in a small minority of ARVD/C patients. Additional ECG criteria proposed in this study included (a) prolonged terminal activation duration, an indicator of activation delay; (b) VT with LBBB morphology and superior axis; and (c) multiple different VT morphologies. These criteria were met in 30 (71%), 28 (67%), and 37 (88%) ARVD/C patients, respectively, and in one control patient (P < 0.001). Electrophysiologic studies contributed importantly to yield different VT morphologies. Pathogenic plakophilin-2 mutations were identified in 25 (60%) of ARVD/C patients and in none of the controls. In ARVD/C patients, parameters measured were not significantly different between mutation carriers and noncarriers, except for negative T waves in V1-3, occurring more frequently in patients with mutation.

CONCLUSIONS

The proposed additional criteria are specific for ARVD/C and more sensitive than the current TFC. Therefore, adding the newly proposed criteria to current TFC could improve ARVD/C diagnosis, independent of DNA analysis.

Authors+Show Affiliations

Department of Cardiology, University of Medical Center Utrecht, Utrecht, The Netherlands. m.g.p.j.cox@umcutrecht.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18373594

Citation

Cox, Moniek G P J., et al. "Activation Delay and VT Parameters in Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy: Toward Improvement of Diagnostic ECG Criteria." Journal of Cardiovascular Electrophysiology, vol. 19, no. 8, 2008, pp. 775-81.
Cox MG, Nelen MR, Wilde AA, et al. Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. J Cardiovasc Electrophysiol. 2008;19(8):775-81.
Cox, M. G., Nelen, M. R., Wilde, A. A., Wiesfeld, A. C., van der Smagt, J. J., Loh, P., Cramer, M. J., Doevendans, P. A., van Tintelen, J. P., de Bakker, J. M., & Hauer, R. N. (2008). Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. Journal of Cardiovascular Electrophysiology, 19(8), 775-81. https://doi.org/10.1111/j.1540-8167.2008.01140.x
Cox MG, et al. Activation Delay and VT Parameters in Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy: Toward Improvement of Diagnostic ECG Criteria. J Cardiovasc Electrophysiol. 2008;19(8):775-81. PubMed PMID: 18373594.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. AU - Cox,Moniek G P J, AU - Nelen,Marcel R, AU - Wilde,Arthur A M, AU - Wiesfeld,Ans C, AU - van der Smagt,Jasper J, AU - Loh,Peter, AU - Cramer,Maarten J, AU - Doevendans,Pieter A, AU - van Tintelen,J Peter, AU - de Bakker,Jacques M T, AU - Hauer,Richard N W, Y1 - 2008/03/26/ PY - 2008/4/1/pubmed PY - 2008/10/31/medline PY - 2008/4/1/entrez SP - 775 EP - 81 JF - Journal of cardiovascular electrophysiology JO - J Cardiovasc Electrophysiol VL - 19 IS - 8 N2 - INTRODUCTION: Desmosomal changes, electrical uncoupling, and surviving myocardial bundles embedded in fibrofatty tissue are hallmarks of activation delay in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Currently, generally accepted task force criteria (TFC) are used for clinical diagnosis. We propose additional criteria based on activation delay and ventricular tachycardia (VT) to improve identification of affected individuals. METHODS AND RESULTS: Activation delay and VT-related 12-lead electrocardiographic (ECG) criteria were studied, while off drugs, in 42 patients with proven ARVD/C according to TFC, and 27 controls with idiopathic VT from the RV outflow tract. Two of three measured TFC could only be identified in a small minority of ARVD/C patients. Additional ECG criteria proposed in this study included (a) prolonged terminal activation duration, an indicator of activation delay; (b) VT with LBBB morphology and superior axis; and (c) multiple different VT morphologies. These criteria were met in 30 (71%), 28 (67%), and 37 (88%) ARVD/C patients, respectively, and in one control patient (P < 0.001). Electrophysiologic studies contributed importantly to yield different VT morphologies. Pathogenic plakophilin-2 mutations were identified in 25 (60%) of ARVD/C patients and in none of the controls. In ARVD/C patients, parameters measured were not significantly different between mutation carriers and noncarriers, except for negative T waves in V1-3, occurring more frequently in patients with mutation. CONCLUSIONS: The proposed additional criteria are specific for ARVD/C and more sensitive than the current TFC. Therefore, adding the newly proposed criteria to current TFC could improve ARVD/C diagnosis, independent of DNA analysis. SN - 1540-8167 UR - https://www.unboundmedicine.com/medline/citation/18373594/Activation_delay_and_VT_parameters_in_arrhythmogenic_right_ventricular_dysplasia/cardiomyopathy:_toward_improvement_of_diagnostic_ECG_criteria_ L2 - https://doi.org/10.1111/j.1540-8167.2008.01140.x DB - PRIME DP - Unbound Medicine ER -