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(Circulation. 2004;110:1527-1534.)
© 2004 American Heart Association, Inc.

Arrhythmia/Electrophysiology

Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy According to Disease Severity

A Need to Broaden Diagnostic Criteria

Khurram Nasir, MD, MPH; Chandra Bomma, MD; Harikrishna Tandri, MD; Ariel Roguin, MD, PhD; Darshan Dalal, MD, MPH; Kalpana Prakasa, MD; Crystal Tichnell, MGC; Cynthia James, PhD; Phillip Jspevak, MD; Frank Marcus, MD; Hugh Calkins, MD

From the Department of Medicine (K.N., C.B., H.T., A.R., D.D., K.P., C.T., C.J., P.S., H.C.), The Johns Hopkins University, Baltimore, Md, and Sarver Heart Disease (F.M.), The University of Arizona Health Sciences Center, Tucson, Ariz.

Correspondence to Hugh Calkins, MD, Carnegie 592, The Johns Hopkins Hospital, 600, N Wolfe St, Baltimore, MD 21287. E-mail hcalkins{at}jhmi.edu

Received December 17, 2003; de novo received February 28, 2004; accepted May 20, 2004.

Background— The purpose of this study was to systematically study diagnostic and prognostic electrocardiographic (ECG) characteristics of arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C).

Methods and Results— The patient population included 50 patients with ARVD/C (27 males, 23 females; mean age 38±15 years). We also analyzed the ECG of 50 age- and gender-matched normal control subject and 28 consecutive patients who presented with right ventricular outflow tract (RVOT) tachycardia. Right bundle-branch block (RBBB) was present in 11 patients (22%). T-wave inversions in V₁ through V₃ were observed in 85% of ARVD/C patients in the absence of RBBB compared with none in RVOT and normal controls, respectively (P<0.0001); epsilon waves were seen in 33%, and a QRS duration 110 ms in V₁ through V₃ was present in 64% of patients. Among those without RBBB, our newly proposed criterion of "prolonged S-wave upstroke in V₁ through V_3" 55 ms was the most prevalent ECG feature (95%) and correlated with disease severity and induction of VT on electrophysiological study. This feature also best distinguished ARVD/C (diffuse and localized) from RVOT.

Conclusions— A prolonged S-wave upstroke in V₁ through V₃ is the most frequent ECG finding in ARVD/C and should be considered as a diagnostic ECG marker.

Key Words: cardiomyopathy • diagnosis • electrocardiography

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