Published Online
on July 7, 2009

A more recent version of this article appeared on October 1, 2009

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Original Article

New ECG Criteria in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Moniek G.P.J. Cox^1,9; Jasper J. van der Smagt²; Arthur A.M. Wilde³; Ans C.P. Wiesfeld⁴; Douwe E. Atsma⁵; Marcel R. Nelen²; Luz-Maria Rodriguez⁶; Peter Loh²; Maarten J. Cramer²; Pieter A. Doevendans²; J Peter van Tintelen⁷; Jacques M.T. de Bakker⁸ and Richard N.W. Hauer²

¹ Univ Med Ctr & Interuniversity Cardio Instit of the Netherlands, Utrecht, The Netherlands;
² University Medical Center Utrecht, Utrecht, The Netherlands;
³ Academic Medical Center, Amsterdam, The Netherlands;
⁴ University Medical Center Groningen, University of Groningen, Groningen, The Netherlands;
⁵ University Medical Center Leiden, Leiden, The Netherlands;
⁶ Maastricht University Hospital, Maastricht, The Netherlands;
⁷ University Medical Center Groningen, University of Groningen, Groningen;
⁸ Univ Med Ctr & Academic Med Ctr & Interuniversity Cardio Inst, The Netherlands

⁹ E-mail: m.g.p.j.cox{at}umcutrecht.nl

Background—Desmosomal changes, electrical uncoupling and surviving myocardial bundles in fibrofatty tissue characterize arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Resultant activation delay is pivotal for reentry, and thereby ventricular tachycardia (VT). Current Task Force Criteria (TFC) for diagnosis have limited sensitivity. The aim of this study was to assess the diagnostic value of additional criteria on activation delay and VT to improve identification of affected individuals.

Methods and Results—ECG criteria were studied, while off drugs, in 50 index patients with proven ARVD/C according to TFC (TFC4 points) and 33 patients with probable ARVD/C (TFC3 points), being 21 index patients and 12 family members of proven ARVD/C patients. Newly proposed additional criteria are: 1) prolonged Terminal Activation Duration (TAD) in V1-3, an indicator of activation delay, 2) VT with LBBB morphology and superior axis, and 3) multiple different VT morphologies. All index patients were screened for mutations in ARVD/C related genes encoding desmosomal proteins. Altogether, 23 of 33 (70%) TFC3 patients would fulfill ARVD/C diagnosis when newly proposed criteria are applied additionally to current TFC. VT with LBBB morphology and superior axis or multiple VT morphologies were recorded in 12 and 9 of 33 TFC3 patients respectively, being all index patients. When applying prolonged TAD additionally to TFC on depolarization/conduction abnormalities, 14 (42%) TFC3 patients fulfill ARVD/C diagnosis. Results were not significantly different between mutation carriers and non-carriers.

Conclusions—Adding the newly proposed criteria to current TFC for ARVD/C will improve identification of affected individuals importantly, independent of outcome of DNA analyses.

Key Words: cardiomyopathy • diagnosis • electrocardiography • tachycardia • Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy