This Article Full Text Full Text (PDF) All Versions of this Article: 2/5/524    most recent CIRCEP.108.832519v1 Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Google Scholar Articles by Cox, M. G.P.J. Articles by Hauer, R. N.W. PubMed PubMed Citation Articles by Cox, M. G.P.J. Articles by Hauer, R. N.W. Related Collections Clinical genetics Electrocardiology Arrhythmias, clinical electrophysiology, drugs

Original Articles

New ECG Criteria in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Moniek G.P.J. Cox, MD; Jasper J. van der Smagt, MD; Arthur A.M. Wilde, MD, PhD; Ans C.P. Wiesfeld, MD, PhD; Douwe E. Atsma, MD, PhD; Marcel R. Nelen, PhD; Luz-Maria Rodriguez, MD, PhD; Peter Loh, MD, PhD; Maarten J. Cramer, MD, PhD; Pieter A. Doevendans, MD, PhD; J. Peter van Tintelen, MD, PhD; Jacques M.T. de Bakker, PhD and Richard N.W. Hauer, MD, PhD

From the Departments of Cardiology (M.G.P.J.C., P.L., M.J.C., P.A.D., J.M.T.d.B., R.N.W.H.) and Medical Genetics (J.J.v.d.S., M.R.N.), University Medical Center Utrecht, Utrecht, The Netherlands; the Department of Clinical Cardiology (A.A.M.W., J.M.T.d.B.), Academic Medical Center, Amsterdam, The Netherlands; the Departments of Cardiology (A.C.P.W.) and Genetics (J.P.v.T.), University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; the Department of Cardiology, University Medical Center Leiden (D.E.A.); the Department of Cardiology, Maastricht University Medical Center, Maastricht (L.-M.R.), The Netherlands; and the Interuniversity Cardiology Institute of The Netherlands (M.G.P.J.C., J.M.T.d.B.).

Correspondence to Moniek G.P.J. Cox, MD, Department of Cardiology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands. E-mail m.g.p.j.cox{at}umcutrecht.nl

Received November 3, 2008; accepted July 6, 2009.

Background— Desmosomal changes, electric 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 (TFC 4 points) and 33 patients with probable ARVD/C (TFC 3 points, or TFC3), being 21 index patients and 12 family members of proven ARVD/C patients. Newly proposed additional criteria are (1) prolonged terminal activation duration in V₁-V₃, an indicator of activation delay, (2) VT with left bundle-branch block morphology and superior axis, and (3) multiple VT morphologies. All index patients were screened for mutations in ARVD/C-related genes encoding desmosomal proteins. Altogether, 23 of 33 (70%) TFC3 patients fulfilled ARVD/C diagnosis when newly proposed criteria were applied additionally to current TFC. VT with left bundle-branch block morphology and superior axis or multiple VT morphologies were recorded in 12 and 9 of 33 TFC3 patients, respectively, all being index patients. When applying prolonged terminal activation duration additionally to TFC on depolarization/conduction abnormalities, 14 (42%) TFC3 patients fulfilled ARVD/C diagnosis. Results were not significantly different between mutation carriers and noncarriers.

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 • electrocardiography • diagnosis • ventricular tachycardia • arrhythmogenic right ventricular dysplasia

---

 

CLINICAL PERSPECTIVE

Dr Rodriguez is deceased.