Background: The use of electrocardiographic (ECG) depolarization and repolarization criteria plays a large role in the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Different ECG algorithms should be analyzed in making the diagnosis of ARVD/C with the use of normal and modified recording techniques.
Methods: In a cohort of 343 patients (210 men and 133 women; mean age, 46.0 +/- 13.7 years) meeting the Task Force of the Working Group on Myocardial and Pericardial Diseases of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology diagnostic criteria for ARVD/C, the value of different ECG criteria (eg, localized right precordial QRS prolongation defined as QRS duration in (V1+V2+V3)/(V4+V5+V6) of 1.2 or higher, right precordial QRS prolongation with QRS in V1-3 of 110 milliseconds or higher, epsilon potentials in the right precordial leads, S-wave upstroke in V1-3 of 55 milliseconds or higher, and right precordial T-wave inversions) was analyzed with the use of a normal recording technique and a highly amplified and modified recording technique (n = 207) at a paper speed of 50 mm/s. Fifty-two phenotypically and genotypically unaffected individuals identified by systematic screening in 24 families (30 men; mean age, 42.4 +/- 8.3 years) were treated as control subjects.
Results: In the normal as well as highly amplified and modified recording techniques, the incidence of localized right precordial QRS prolongation was 98% (100%), that of QRS in V1-3 of 110 milliseconds or higher was 75% (80%), that of prolonged right precordial S-wave upstroke was 84% (60%), that of epsilon potentials was 23% (77%), and that of right precordial T-wave inversions was 55%. Four of 6 patients without the phenomenon of localized right precordial QRS prolongation with the use of the normal recording technique had a prolonged S-wave upstroke of 55 milliseconds or higher. In the control group, localized right precordial QRS prolongation, QRS in V1-3 of 110 milliseconds or higher, and epsilon potentials could not be identified. An S-wave upstroke of 55 milliseconds or higher was present in 2 of 3 cases, and T-wave inversions were found in 3.
Conclusions: Electrocardiographic depolarization criteria for ARVD/C analyzed in this large cohort of patients meeting the International Society and Federation of Cardiology/European Society of Cardiology criteria presented with high sensitivity and specificity in comparison with those in the control group of phenotypically and genotypically unaffected individuals defined by systematic screening in 24 families with ARVD/C. The incidence of right precordial T-wave inversions was much lower, indicating that not only patients with overt right ventricular dilatation and dysfunction were included. Electrocardiographic algorithms, including localized right precordial QRS prolongation, prolonged S-wave upstroke, and epsilon potentials, with the use of the normal recording technique and the amplified and modified recording technique at a paper speed of 50 mm/s contribute significantly to the noninvasive diagnosis of ARVD/C.