Until recently it was generally thought that early repolarization is benign. But a recent article
in the NEJM (Haissaguerre et al.) suggests that some persons with early repolarization may be
at risk of life-threatening ventricular tachyarrhythmia. Unexplained syncope or sudden death
occurs mostly during sleep. However, some cases of cardiac arrest during exertion have been
reported.
We report the case of a 39 year-old black African male with early repolarization pattern on
electrocardiogram who regularly experienced dizziness (and one episode of transient loss of
consciousness) exclusively while exercising. Detailed examination was normal. Under quinidine
therapy, he experienced no further episodes. Increasingly reported cases of cardiac arrest
in Africans, and significant prevalence of early repolarization in this population, have to be
taken into account since the Haissaguerre et al. report. Further evidence of the lethal consequences
of this syndrome are needed, bearing in mind that diagnostic tools for life-threatening
arrhythmias are often scarce in sub-Saharan Africa

Amara, Walid

Bonny, Aimé

Ditah, Ivo

Frank, Robert

Hamdaoui, Brahim

Heuzey, Jean-Yves Le

English

Via Medica Journals

www.cardiologyjournal.org 259CASE REPORTCardiology Journal2009, Vol. 16, No. 3, pp. 259–263Copyright © 2009 Via MedicaISSN 1897–5593ôAddress for correspondence: Aimé Bonny, Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75908 Paris, France,tel: +33 658 19 54 09, fax: +33 498 31 009, e-mail: aimebonny@yahoo.frReceived:  7.12.2008 Accepted: 4.01.2009Early repolarization electrocardiography patternwith unexplained syncope during training ina young black African non-elite athlete:An accidental finding?Aimé Bonny1, 2, Ivo Ditah3, Walid Amara4, Brahim Hamdaoui5,Robert Frank2, Jean-Yves Le Heuzey11Hôpital Européen Georges Pompidou, Service de Cardiologie, Paris, France2Hôpital Salpetrière, Institut de Cardiologie, Unité de Rythmologie, Paris, France3Institute of Public Health, School of Medicine, Health Policy and Practice,University of East Anglia, Norwich, United Kingdom4Centre Hospitalier Le Raincy-Montfermeil, Service de Cardiologie, Montfermeil, France5Hôpital Henri Mondor, Service de Cardiologie, Créteil, FranceAbstractUntil recently it was generally thought that early repolarization is benign. But a recent articlein the NEJM (Haissaguerre et al.) suggests that some persons with early repolarization may beat risk of life-threatening ventricular tachyarrhythmia. Unexplained syncope or sudden deathoccurs mostly during sleep. However, some cases of cardiac arrest during exertion have beenreported.We report the case of a 39 year-old black African male with early repolarization pattern onelectrocardiogram who regularly experienced dizziness (and one episode of transient loss ofconsciousness) exclusively while exercising. Detailed examination was normal. Under quini-dine therapy, he experienced no further episodes. Increasingly reported cases of cardiac arrestin Africans, and significant prevalence of early repolarization in this population, have to betaken into account since the Haissaguerre et al. report. Further evidence of the lethal conse-quences of this syndrome are needed, bearing in mind that diagnostic tools for life-threateningarrhythmias are often scarce in sub-Saharan Africa. (Cardiol J 2009; 16, 3: 259–263)Key words: early repolarization syndrome, ventricular arrhythmias, syncopeIntroductionTransient loss of consciousness (TLOC) isa common disease with a cumulative lifetime inci-dence of 35% [1]. TLOC is usually due to either se-izure or syncope. Syncope during exertion is oftenserious and of cardiac origins, while syncope imme-diately after stopping exercise is generally neurally--mediated (reflex syncope) and benign [2].Known cardiac causes of syncope are structu-ral and non-structural. Potential structural cardiaccauses of exercise-related syncope are coronary260Cardiology Journal 2009, Vol. 16, No. 3www.cardiologyjournal.orgdisease, aortic stenosis (AS), hypertrophic cardio-myopathy (HCM) and arrhythmogenic right ventri-cular dysplasia/cardiomyopathy (ARVD/C). Electri-cal heart diseases such as long QT syndrome(LQTS), short QT syndrome (SQTS), catecholami-nergic polymorphic ventricular tachycardia (CPVT)are common as causes of non-structural exercisesyncope. When these pathologies are excluded aspotential causes of unexplained syncope or cardiacarrest during exertion, early repolarization patternin electrocardiogram (ECG) should be taken intoaccount in light of  the Haissaguerre et al. report [3].We report an unusual feature of exertion syncopewith early repolarization variant (ERPV).Case reportA 39 year-old black African male lost conscio-usness while he was playing football. Eyewitnes-ses (other players) reported a true absence of con-sciousness with eyeball revulsion and tongue biting.He spontaneously recovered consciousness aftera short time. The eyewitnesses reported no post-event disorientation. The patient remembered thatbefore he lost consciousness he experienced dizzi-ness about 15 minutes into the game and was for-ced to bend over, as had happened during previousepisodes. In the three months before syncope heregularly experienced sudden weakness and dizzi-ness during soccer games. He never had such symp-toms at rest and there was no history of suddendeath in first degree relatives. He regularly playedsoccer one hour a week. When he was aged 29, forrecruitment purposes he was subject to an ECGshowing early repolarization in apicolateral (V3–V6,I, aVL) and inferior (II, aVF) leads; mimickingacute ischemic disease. One year before the cardiacarrest, a systematic check-up with ECG confirmedmarked ERPV (Fig. 1). After recovering conscious-ness, the patient underwent detailed cardiac andneurological evaluation: clinical examination inclu-ding carotid sinus massage, electroencephalogram,ambulatory ECG recording, brain magnetic reso-nance (MRI) and coronary angiography were withinnormal limits. The ECG displayed ERPV. Two--dimensional echocardiography suspected apical leftventricular non-compaction, but cardiac MRI exc-luded this diagnosis, as well as ARVD. AS and HCMwere also excluded. Ventricular ectopic beats did notappear during exercise stress testing, which inducednormalization of repolarization (Fig. 2). The tilt-testwas not performed and the patient gave up playingsoccer. Almost two years later, after publication ofHaissaguerre et al. [3] we again contacted the pa-tient and suggested that we study him for arrhythmiavulnerability by programmed ventricular stimula-tion. Unfortunately he refused. He agreed to takequinidine hydrochloride after counselling. ECGbefore treatment showed a prominent J point withupward concavity ST segment elevation in apicola-teral (V3–V6, I, aVL) and inferior (II, aVF) leads(Fig. 3). Quinidine (300 mg twice daily) consisten-tly reduced the early repolarization pattern after twoweeks (Fig. 3). Under quinidine therapy, the patienthas recently started playing soccer again oncea week, and had no complaints at three monthsfollow up.Diagnostic criteriaElectrocardiographic features of ERPV havebeen recently described [4, 5]. Although these fe-atures are quite diverse, they have one factor inFigure 1. One year before loss of consciousness, ECG displayed prominent J point in anterolateral (V2–V6, I, aVL)and inferior (II, aVF) leads. An elevation of J-STT junction (the so-called Osborn wave) of at least 0.1 mV from baselinemanifested as QRS slurring (I, II, aVL, aVF, V6) or notching (V2–V5) with upward concavity. Early R-wave progression inprecordial leads with increased voltage in V4–V6 than standard leads. QTc interval is relatively short: 390 ms.261Aimé Bonny et al., Early repolarization pattern with unexplained syncopewww.cardiologyjournal.orgFigure 2. Exercise stress testing (Bruce protocol). The prominent J point with slurring (notching in V3) descending leg ofthe QRS complex (I, II, V4–V6) at the rest (0:01, HR = 92/min) disappeared during effort (7:58, HR = 187/min). Arrowspoint out where the slur (notch) appears before the test, during effort and the recovered period; HR — heart rate.Figure 3. A. Before quinidine treatment — ECG shows early repolarization as QRS slurring in inferolateral (I, II, aVL,V4–V6) and prominent J point followed by notching ST elevation in V3. Lead V4 displays maximal R wave voltage.Intrinsicoid deflection is rapid in leads displaying early repolarization; B. Under quinidine therapy — after two weeks(300 mg twice daily): prominent J wave, slurring J-STT elevation and ST elevation with upward concavity were significan-tly reduced in leads V4–V6 and II. ‘Western saddle’ pattern was concealed in lead V3.AB262Cardiology Journal 2009, Vol. 16, No. 3www.cardiologyjournal.orgcommon: slurring or notching that produces a po-sitive hump in QRS-ST junction: a prominent J waveresembling an Osborn wave. This aspect is foundat the junction of the end of the QRS complex andthe beginning of the ST segment. The lead withprominent QRS-ST junction of at least 0.1 mV com-monly shows ST segment elevation displayingupward concavity. QRS voltage is increased in con-cerned leads, mostly in V4 and V5. An asymmetricQRS complex with slurring and a reduced slopeangle of the ascending positive R wave, an extre-mely rapid intrinsicoid deflection of descending partof QRS wave and a prominent J wave are commonsigns. The T wave can be inverted or biphasic.Dynamic changes may occur in the width andheight of the wave. The J wave may show circadianchanges which may not always be present, and usu-ally disappear during exercise. The common loca-tion of early repolarization pattern are leads I, II,III, VF, VL, V4 to V6 but right precordial leads (V2,V3) may also show abnormalities [4]. The ERPVwhich includes the above mentioned ECG featuresand a history of unexplained syncope or cardiac ar-rest due to ventricular fibrillation (VF) seems tohave the following properties: the magnitude ofJ-point elevation ≥ 1.5 mm, the QRS width and theQT interval both may be shorter than normal.Quinidine use, exercise testing, or the infusion ofisoproterenol consistently reduces or eliminatesearly repolarization.DiscussionSyncope while exercising, but not on cessationof exercise, is very often cardiogenic and threemechanisms are often involved: mechanicalobstruction when AS or obstructive HCM is present;tachyarrhythmias due to coronary disease, HCM,ARVD, electrical heart diseases such as LQTS,SQTS, CPVT and bradyarrhythmias due to paroxy-smal heart block. Some authors report anecdotaldata of syncope and sudden cardiac death withprominent J-waves [6–11]. These isolated casereports have mentioned such changes in the QRS-STjunction in men with idiopathic VF. However, thelack of information from large multicenter studieshas remained a drawback. Haissaguerre et al. [3]were the first to demonstrate with high accuracythe possible link between early repolarization pat-tern and unexplained syncope or sudden cardiacdeath due to VF. In their work, the link betweenthis ECG pattern and malignant arrhythmias hasbeen supported by both the accentuation of earlyrepolarization (increase of a prominent J point)before the onset of arrhythmia, and the origin oftriggering beats from the region of early repolari-zation. Syncope or cardiac arrest occurred mostly atrest, but some patients experienced it during exertion.Quinidine diminished the ECG pattern and elimi-nated the recurrence of arrhythmia in 100% (4/4)of patients [3].In fact, quinidine has been shown to restoretransmural electrical homogeneity and abort arrhy-thmic activity in this condition. Gussak and Antze-levitch [12] and Yan and Antzelevitch [13] have de-monstrated the role of quinidine in restoring trans-mural dispersion of repolarization by reducingoutward potassium Ito current, leading to normalizethe J point (the so-called Osborn wave). Syncopeduring exertion is often serious, and of cardiacorigins, while syncope immediately after stoppingexercise is generally benign and of vasovagal (com-mon faint) or situational causes. Although we havenot proved the arrhythmia susceptibility of this pa-tient, many of the facts could point to it: clinicalevent while exercising, normal carotid sinus mas-sage, exclusion of other causes of exercise-relatedsyncope as AS, obstructive HCM, coronary disease,ARVD, LQTS, SQTS and CPVT. However, we as-sumed that tilt-testing would definitively help toexclude neurally-mediated syncope. Unexplainedsyncope during exertion in this young adult withoutstructural heart disease, negative extra-cardiacevaluation, early repolarization features in the ECG,consistent reduction of early repolarization duringexercise stress testing and a possible efficacy onjunctional changes of quinidine after three monthsof follow up are likely features of the syndromedescribed by Haissaguerre et al. [3]. Moreover,syncope in a patient with the prominent J wave hasalready been described [6].Our patient underwent a detailed cardiac, neu-rological and psychiatric evaluation without any fin-ding apart from early ERPV in ECG (Fig. 1): slur-ring and notching of J-STT elevation with upwardconcavity, increased QRS voltage in V4 and V5,asymmetric QRS complex with a reduced slopeangle of the ascending R wave, an extremely fastintrinsicoid deflection of descending R wave, a pro-minent J wave and different morphologic forms andspatial distributions of ST elevation. This patternof repolarization has been overlooked until recentfindings suggesting a possible link between ERPVand idiopathic ventricular arrhythmia were repor-ted [3], leading to the proposition of an electrophy-siological (EP) study and quinidine treatment to ourpatient almost two years later. This case report isvery unusual in so far as he combined both features263Aimé Bonny et al., Early repolarization pattern with unexplained syncopewww.cardiologyjournal.orgof cardiac and neutrally-mediated mechanisms. Un-completed diagnostic evaluation including tilt-te-sting and EP study look like the situation which iscommon in sub-Saharan Africa. Cardiac arrest is in-creasingly reported in black Africans [14] and epi-demiological studies have shown that ERPV is moreprevalent in dark-skinned persons [15]. For thesereasons, in the future, clinicians will be faced withthe crucial issue of deciding whether unexplainedsyncope in  a patient with ERPV was an arrhyth-mic event or not. This decision is even more diffi-cult in sub-Saharan Africa where diagnostic tools forarrhythmic sudden death and syncope such as Hol-ter monitoring, external or internal loop recorders,tilt-testing, EP study and cardiac imaging techniquesare limited.As the prevalence of ECG features of earlyrepolarization is fairly high in the general popula-tion without affecting life expectancy for a signifi-cant proportion, the challenge is to distinguish bothgroups, with and without risk markers of suddencardiac death [15]. Although our understanding ofatypical patterns of ECG is steadily improving, weneed to document the accuracy of non-invasivetests and signs of junctional changes in inferolateralleads for which ventricular ectopic activity wouldbe likely. Thus, countries with low incomes, insuf-ficient medical facilities and high prevalence ofERPV will have to address  the problem of  loss ofconsciousness in subjects having early repolarisa-tion ECG pattern.Limitations of the studyAlthough this report did not show the occur-rence of ventricular tachyarrhythmia leading to syn-cope, detailed history taking, physical examinationand additional investigations were consistent withHaissaguerre’s description of this syndrome.However, we are aware that being event-free for threemonths of follow-up on quinidine may be random;ECG repolarization changes in transitional chestlead (V3) could be due to electrode placement andgenerally the pattern of J-STT can also be dynamic,changing in degree from one recording to the next.ConclusionsThis study looks at ERPV and unexplainedsyncope that could be caused by a self-terminatingidiopathic VF and which should alert the attentionof clinicians to this entity. Increasingly reportedcases of cardiac arrest in Africans and a significantprevalence of ERPV in this population have to betaken into account.AcknowledgementsThe authors do not report any conflict of inte-rest regarding this work.References1. Ganzeboom KS, Mairuhu G, Reitsma JB, Linzer M, Wieling W,van Dijk N. Lifetime cumulative incidence of syncope in thegeneral population: a study of 549 Dutch subjects aged 35–60years. J Cardiovasc Electrophysiol, 2006; 17: 1172–1176.2. Brignole M, Alboni P, Benditt DG et al. Task force on syncope,European Society of Cardiology. Guidelines on management(diagnosis and treatment) of syncope: Update 2004. Europace,2004; 6: 467–537.3. Haïssaguerre M, Derval N, Sacher F et al. Sudden cardiac arrestassociated with early repolarization. N Engl J Med, 2008; 358:2063–2065.4. Boineau JP. The early repolarization variant: An electrocardio-graphic enigma with both QRS and J-STT anomalies. J Electro-cardiol, 2007; 40: 3 (e1–e10).5. Riera Pérez RA, Uchida Hiroshi A, Schapachnik E et al. Earlyrepolarization variant: Epidemiological aspects, mechanism, anddifferential diagnosis. Cardiol J, 2008; 1: 4–16.6. Bjerregaard P, Gussak I, Kotar SL, Gessler JE, Janosik D. Re-current syncope in a patient with prominent J-wave. Am Heart J,1994; 127: 1426–1430.7. Klasky AL, Oehm R, Cooper RA, Udalstova N, Armstrong MA.The early repolarization normal variant electrocardiogram: Cor-relates and consequences. Am J Med, 2003; 115: 171–177.8. Mehta M, Jain AC, Mehta A. Early repolarization. Clin Cardiol,1999; 22: 59–65.9. Letsas KP, Efremidis M, Pappas LK et al. Early repolarizationsyndrome: Is It always benign? Int J Cardiol, 2007; 114: 390––392.10. Garg A, Finneran W, Feld KF. Familial sudden death associatedwith terminal QRS abnormality on surface 12-lead electrocardiogramin the index case. J Cardiovasc Electrophysiol, 1998; 9: 642–647.11. Nam GB, Kim YH, Antzelevitch C. Augmentation of J waves andelectrical storms in patients with early repolarization. N EnglJ Med, 2008; 358: 2078–2079.12. Gussak I, Antzelevitch C. Early repolarization syndrome: Clini-cal characteristics and possible cellular and ionic mechanisms.J Electrocardiol, 2000; 33: 299–309.13. Yan GX, Antzelevitch C. Cellular basis for the electrocardio-graphic J wave. Circulation, 1996; 93: 372–379.14. Bonny A, Tonet J, Fontaine G et al. Brugada syndrome in pureblack Africans. J Cardiovasc Electrophysiol, 2008; 19: 421–426.15. Wellens HJ. Early repolarization revisited. N Engl J Med, 2008;358: 2063–2065.

Early repolarization electrocardiography pattern with unexplained syncope during training in a young black African non-elite athlete: An accidental finding?

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Early repolarization electrocardiography pattern with unexplained syncope during training in a young black African non-elite athlete: An accidental finding?

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