Europace (2008) 10, 1447–1449 doi:10.1093/europace/eun279

SHORT COMMUNICATION Early repolarization phenomenon in arrhythmogenic right ventricular dysplasia– and sudden due to ventricular fibrillation Downloaded from https://academic.oup.com/europace/article/10/12/1447/464834 by guest on 26 September 2021

Stefan Peters1* and Daniela Selbig2

1Asklepios Harzkliniken GmbH Goslar, and Intensive Care, Ko¨sliner Street 12, 38642 Goslar, Germany; and 2Klinikum Quedlinburg, Cardiology, Quedlinburg, Germany

Received 18 July 2008; accepted after revision 15 September 2008; online publish-ahead-of-print 22 October 2008

KEYWORDS The case of a 26-year-old male with sudden cardiac arrest due to ventricular fibrillation and the final Arrhythmogenic right diagnosis of arrhythmogenic right ventricular dysplasia–cardiomyopathy (ARVD/C) and initial early repo- ventricular dysplasia- larization phenomenon is presented in detail. An additional analysis of early repolarization in additional cardiomyopathy; 359 patients with ISFC/ESC diagnostic criteria of ARVD/C revealed a frequency within the threshold in Early repolarization; healthy volunteers with 3% in isolated lateral leads and 7% in inferolateral leads. The high frequency of Late depolarization electrocardiographic early repolarization limited to inferior leads (22%) is probably due to late depolar- ization and represents an already reported typical feature of ARVD/C.

Introduction resuscitation, standard ECG still reveals early repolarization in inferior leads and right pre-cordial inversions Recently, early repolarization phenomenon was identified as (Figure 1, right panel). an ECG feature associated with idiopathic ventricular fibril- 1 Right ventricular angiography revealed a grossly enlarged lation. Arrhythmogenic right ventricular dysplasia–cardio- right with reduction of global function (ejection myopathy (ARVD/C) was excluded in these cases by right fraction 45%) and segmental sacculations in the inferior ventricular angiography or cardiac magnetic resonance and apical segments. The electrophysiological study could imaging. Late potential analysis was not different in patients provoke non-sustained ventricular flutter. Implantable car- with and without early repolarization phenomenon. dioverter defibrillator implantation was performed. According to the literature, early repolarization phenom- enon does not belong to the classical electrocardiographic criteria of ARVD/C. Analysis of early repolarization phenomenon We describe a case of sudden cardiac arrest due to ventri- in ARVD/C cular fibrillation in a patient with ARVD/C and early repolar- ization phenomenon. Furthermore, early repolarization is We reviewed the ECG of 359 patients (174 males, mean age analysed retrospectively in additional 359 patients with 47.3 + 13.7 years) with ARVD/C according to the ISFC/ESC the diagnosis of ARVD/C according to IFSC/ESC diagnostic diagnostic criteria. ECGs were recorded at 50 mm/s paper criteria. speed, 10 mm/mV amplitude, and 40 and 50 Hz filtering technique. Early repolarization was defined as an elevation of the QRS–ST junction (J point) of at least 1 mm (0.1 mV) Case report in at least two leads,2,3 either as QRS slurring (a smooth tran- A 26-year-old male suffered from sudden cardiac arrest due sition from the QRS segment to the ST-segment) or notching to ventricular fibrillation and was successfully resuscitated. (a positive J deflection inscribed on the S wave) in inferior Early repolarization phenomenon in inferolateral leads (leads II, III, and aVF) or lateral leads (I, aVL, and V4–V6). (Figure 1, left panel) was documented. Coronary artery In a total of 112 out of the 359 patients (31%), early repo- disease was excluded. On day 2 after successful larization phenomenon could be found in inferior, lateral, or inferolateral leads. In 79 cases (22%), early repolarization * Corresponding author. Tel: þ49 5321 44 1400. was limited to inferior leads. In isolated lateral leads with E-mail address: [email protected] 10 patients (3%) or both inferolateral leads with 23 patients

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2008. For permissions please email: [email protected]. 1448 S. Peters and D. Selbig Downloaded from https://academic.oup.com/europace/article/10/12/1447/464834 by guest on 26 September 2021

Figure 1 (Left panel) (leads I, II, III, aVR, aVL, aVF from top to bottom) Standard 12-lead ECG of a 26-year-old male patient with ARVD/C after successful resuscitation of ventricular fibrillation with early repolarization phenomenon in leads II, III, aVF, and V4–V6. (Right panel) Standard 12-lead ECG of the same patient on day 2 after resuscitation with persisting early repolarization in leads II, III, and aVF and T wave inversions in V1–V3.

(7%), early repolarization was a rare phenomenon. There Without exclusion of late potentials in inferior leads the was no correlation between the electrocardiographic main question remains whether early repolarization accord- phenomenon of early repolarization and clinical findings ing to electrocardiographic criteria is indeed late depolariz- with regard to sudden cardiac arrest, syncope, sustained ation. In isolated lateral and both inferolateral leads, early ventricular , or no arrhythmic events. repolarization in ARVD/C is within the threshold of early repolarization in apparently healthy adults with a frequency of 7%.5 This interpretation of the reported data is in very Discussion good accordance to the fact that arrhythmic events in ARVD/C patients with and without early repolarization The phenomenon of early repolarization has long been show no significant differences. recognized as a benign ECG variant. Only systematic analysis In conclusion, the case reported in this paper represents of early repolarization in idiopathic ventricular fibrillation the first description of early repolarization with ventricular recently published1 shed a new light on the meaning of fibrillation in ARVD/C. Early repolarization is a rare finding this electrocardiographic feature. in lateral and inferolateral leads of ARVD/C patients not In ARVD/C, early repolarization has not been described different from healthy adults. earlier. In the recently published report of early repolariza- tion in idiopathic ventricular fibrillation, ARVD/C was excluded in all patients by right ventricular angiography or Limitations magnetic resonance imaging. To our best knowledge, the reported case in this paper represents the first description In all cases of electrocardiographic early repolarization of early repolarization with consecutive ventricular fibrilla- phenomenon in patients with typical ARVD/C delayed tion in a typical case of ARVD/C. depolarization cannot be excluded without performance of In the collective of additional 359 patients with ISFC/ESC signal-averaged ECG. Even in the case reported with exten- diagnosis of ARVD/C, the frequency of early repolarization is sive segmental sacculation in the inferior wall of the right as high as in the recently published cohort of ventricular ventricle inferolateral ECG changes might be due to late fibrillation. The main difference, however, is that in the depolarization. majority of cases with ARVD/C, early repolarization is Furthermore, it is difficult to decide whether ventricular limited to inferior leads. Prolongation of the QRS interval fibrillation is due to early repolarization in the case duration in inferior leads due to slurring or notching of the reported. Early repolarization might be a coincidental transition of the QRS complex and the ST-segment was finding in the patient with ARVD/C with probable ventricular already reported as a typical finding of ARVD/C in more tachycardia degenerating into ventricular fibrillation. than 20% of cases and associated with left ventricular abnormalities in the inferior and posterolateral segments.4 Conflict of intereset: none declared. Early repolarization phenomenon in ARVD/C 1449

References 3. Mehta M, Jain AC, Mehta A. Early repolarization. Clin Cardiol 1999;22: 59–65. 1. Haissaguerre M, Derval N, Sacher F, Jesel L, Deisenhofer I, de Roy L et al. 4. Peters S, Tru¨mmel M. Diagnosis of arrhythmogenic right ventricular Sudden cardiac arrest associated with early repolarization. N Engl J Med dysplasia–cardiomyopathy: value of standard ECG revisited. Ann 2008;358:2016–23. Noninvasive Electrocardiol 2003;8:238–45. 2. Klatsky AL, Oehm R, Cooper RA, Udalstova N, Armstrong MA. The early 5. Kui C, Congxin H, Xi W, Yan-hong T, Okella E, Salim M et al. Characteristic repolarization normal variant ECG: correlates and consequences. Am J of the prevalence of J wave in apparently healthy Chinese adults. Arch Med 2003;115:171–7. Med Res 2008;39:232–5. Downloaded from https://academic.oup.com/europace/article/10/12/1447/464834 by guest on 26 September 2021