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REVIEW

Clinical update

Clinical significance of variants of J-points andJ-waves: early repolarization patterns and risk

M. Juhani Junttila1,2*, Solomon J. Sager1, Jani T. Tikkanen2, Olli Anttonen3,

Heikki V. Huikuri2, and Robert J. Myerburg1

1Division of Cardiology (D-39), Miller School of Medicine, University of Miami, PO Box 016960, Miami, FL 33101, USA; 2Department of Internal Medicine, Institute of Clinical

Medicine, University of Oulu, Oulu, Finland; and 3Division of Cardiology, Paijat-Hame Central Hospital, Lahti, Finland

Received 31 October 2011; revised 5 January 2012; accepted 3 April 2012

The variations in the electrocardiographic patterns of J-point elevations, and the complex of J-points and J-waves in early repolarization (ER),

in conjunction with disparities in associated sudden cardiac death (SCD) risk, have lead to a recognition of the need to carefully classify the

spectrum of these observations. Many questions about the pathogenesis of J-wave patterns, and the associated magnitudes of risk, remain

unanswered, especially in regard to the risk implications in certain high-prevalence subpopulations such as athletes, children, and adolescents.

Interest in these electrocardiography (ECG) patterns has grown dramatically in recent years, in large part because of the frequency with

which these patterns are observed on routine ECGs. In this review, we discuss the current knowledge on the prevalence of different J-

point/J-wave patterns and estimates of the magnitude of mortality and SCD risk associated with J-point elevations and J-waves, in what

has become known as ER patterns.- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -

Keywords Early repolarization Brugada syndrome Sudden cardiac death

Introduction

The J-point on the electrocardiographic waveform is historically

defined as the junction between the end of the QRS complex

and the beginning of the ST-segment.1,2 In 1953, Osborn3

described the presence of broad positive deflections originating

from an elevated J-point, induced by experimental hypothermia

and associated with ventricular fibrillation. He considered them

currents of injury. They came to be known as J-waves bearing

his name (Osborn waves) and have become a generally accepted

marker for clinical hypothermia. However, during the same

period of time, others noted less dramatic J-point elevations withconcomitant J-wave deflections, primarily in the anterior leads,

on electrocardiograms recorded from normal young individuals.

This pattern was considered benign, even though the Osborn

J-wave variant had been considered clinically significant at the

same time.4,5 The normal variants in young subjects became

defined by the term early repolarization (ER). The ER pattern is

more prevalent among males, African-Americans, and adolescents,

and it is accentuated by vagal tone and hypothermia.610 In more

recent years, the notion that the J-wave of ER was a universally

benign normal variant, except when induced by exogenous

factors came into question, as data emerged on variants of

J-point elevation and J-waves that were associated with conditions

that carried a risk of sudden cardiac death (SCD). These included

the electrocardiographic patterns observed in the right precordial

leads in the Brugada syndrome, and inferolateral ER or J-waves re-

cently associated with an increased risk of mortality and SCD in

casecontrol and general population studies.1118

The variations in the electrocardiographic patterns of J-point

elevations, and the complex of J-points and J-waves in ER, in con-

junction with disparities in associated SCD risk, have lead to a rec-ognition of the need to carefully classify the spectrum of these

observations.19 Many questions about the pathogenesis of J-wave

patterns, and associated magnitudes of risk, remain unanswered,

especially in regard to the risk implications in certain high-

prevalence subpopulations such as athletes, children, and adoles-

cents. Interest in these electrocardiography (ECG) patterns has

grown dramatically in recent years, in large part because of the fre-

quency with which these patterns are observed on routine ECGs.

* Corresponding author. Tel: +358 8 3156213, Fax: +358 8 3155599, Email: [email protected]

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2012. For permissions please email: [email protected]

European Heart Journal

doi:10.1093/eurheartj/ehs110

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In this review, we discuss the current knowledge on the prevalence

of different J-point/J-wave patterns and estimates of the magnitude

of mortality and SCD risk associated with J-point elevations and

J-waves, in what has become known as ER patterns.

Anterior J-point elevations and

J-waves in the Brugada syndromeThe Brugada syndrome is characterized by a right bundle branch

block pattern with ST-segment elevation and inversion of the ter-

minal part of T-wave in the right precordial leads.18,20 The J-wave

pattern may vary from time to time within the series of ECGs in

individual patients, tends to be accentuated by increased vagal

tone and fever, and can be unmasked or enhanced by the Class I

membrane-active antiarrhythmic drugs, ajmaline, flecainide, and

procainamide.2024 Exercise, catecholamine stimulation, and quini-

dine have been shown to normalize the Brugada ECG pattern, al-

though some conflicting results have been reported between the

Asian and European Brugada syndrome populations.20,21,25 There

is also a considerable male predominance in the incidence of thesyndrome.19

The ECG patterns within the spectrum of the Brugada syn-

drome are classified into three categories (Figure1). J-point eleva-

tions are characteristic of all three patterns of Brugada-associated

ECGs, the distinctions between the patterns reflected primarily in

the J-waveforms following the J-points, and extending into the

ST-segments and T-waves. The Type I pattern, J-point elevation

with a coved J-waveST-segment configuration, is the most specif-

ic and considered the highest risk pattern, especially in symptom-

atic patients. The Type I ECG pattern is the only diagnostic ECG

for the Brugada syndrome.20 Asymptomatic patients with Type I

patterns are also at increased risk compared with the generalpopulation without Type I patterns or compared with those with

Type II patterns, but the magnitude of risk is considerably lower.

Moreover, some ECG patterns are associated with higher risk of

symptoms or life-threatening events in the Brugada syndrome: spe-

cifically, higher J-point elevations, QRS durations .100 ms, and a

prominent r in lead aVR.26 To date, no data suggest that athletes

have a higher prevalence of Brugada ECG patterns, even though

many athletes have ER patterns in the right precordial leads.27

Interestingly, inferolateral ER patterns have also been reportedto be present in as high as 11 15% of Brugada patients and have

been shown to have a strong adverse effect on the prognosis.2830

The prevalence of the diagnostic Brugada ECG is ethnicity-

dependent. Among the Asian population, the prevalence of the

Type I Brugada pattern has been estimated to be 0.4% and in

the European population 00.01%.3135 The annual incidence of

life-threatening arrhythmias has been estimated to be 7.7%

among patients with aborted SCD, 1.9% among patients with

syncope, and 0.5% in asymptomatic subjects.36 Although the inci-

dence of life-threatening arrhythmias in asymptomatic Brugada

patients remains a debated issue,37,38 the majority of life-

threatening or fatal arrhythmias in patients with the Brugada syn-

drome are nocturnal, likely due to the association of vagal tone

with the amplitude of the J-wave.20,39

Although most investigators consider the pathophysiology of

Brugada syndrome-associated J-waves to be regional ER, recent

data suggest the possibility of delayed depolarization in the right

ventricular outflow tract as a contributing mechanism to J-waves,

arrhythmia expression, or perhaps both.4042

Inferolateral J-point elevationsand J-wavesrisk-associated early

repolarization patternsInferolateral ER is characterized by a deflection in the R-wave

descent (slurred pattern) or a positive deflection with a secondary

r wave (notching pattern) in the terminal part of the QRS

complex in at least two inferior (II, III, aVF) leads, in two lateral

(I, aVL, V46) leads, or both. The association between SCD and

inferolateral ER was first observed by Haissaguerre et al.11 in a

casecontrol study of 206 patients who survived ventricular fibril-

lation in the absence of a defined cause (idiopathic ventricular fib-

rillation, IVF) and 412 matched control subjects. Inferolateral ER

was observed in 31% of the IVF group, compared with 5% of the

controls. The IVF patients had significantly greater amplitude of

J-point elevation than controls, and subjects with extreme J-pointelevation (.5 mm) had the highest occurrences of VF episodes.11

Multiple casecontrol studies have confirmed this association and

revealed that inferolateral ER amplitude is strongly associated with

vagal tone and hypothermia.1214,43,44 Additionally, exercise, quini-

dine, and catecholamines abolish the J-wave.8 Two independent

family studies have recently suggested the inheritance of propensity

to ER patterns. In a study from the Framingham Heart Study popu-

lation, the siblings of ER subjects were twice as likely to have ER

than non-ER subjects (OR 2.22, P , 0.05), and in a study on

over 500 British families drawn from a general population

cohort, ER was over two times more likely to occur in children

of the family (OR 2.54, P 0.005) if one of the parents had an

Figure 1 Brugada electrocardiography patterns. Brugada syn-

drome subtypes are shown. (Left) Typical Type I (coved type)

Brugada syndrome electrocardiography pattern, (middle) Type

II (saddleback) Brugada electrocardiography pattern, and (right)

Type III Brugada electrocardiography pattern. Types II and III

have an ascending ST-segment after the J-wave but diagnostic

Type I electrocardiography has a descending ST-segment with

T-wave inversions after the J-wave. Paper speed 50 mm/s, gain

10 mm/mV. Reprinted from Junttila et al. Prevalence and progno-

sis of subjects with Brugada-type electrocardiography pattern in a

young and middle-aged Finnish population. Eur Heart J

2004;25:8748. &Oxford University Press.

M.J. Junttila et al.Page 2 of 6


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ER ECG pattern.45,46 A recent paper also demonstrated the familial

occurrence of the ER pattern in subjects with IVF and ER. 47

The prevalence and prognosis of inferolateral ER has been

studied extensively in three general population studies.1517 The

first was conducted in Finland and included 10 864 middle-aged

subjects representative of the general population who were en-

rolled into a population-based study of coronary heart disease

between 1966 and 1972, with a mean follow-up of 30+11years. The prevalence of inferolateral ER recorded on ECGs at

entry was 5.8% (inferior ER 3.5%, lateral ER 2.4%, or both 0.1%).

Inferior ER was associated with an increased risk of cardiac mortal-

ity [risk ratio (RR) 1.28, P 0.03], and inferior ER patterns with

J-point elevations .0.2 mV was associated with cardiac mortality

(RR 2.98, P , 0.001) and sudden arrhythmic death (RR 2.92, P

0.01). In the same population, QTc durations .440 ms in males

and 460 ms in females were associated with a smaller magnitude

of increased risk for cardiac mortality (RR 1.20, P 0.03), as was

the SokolowLyon voltage criteria for left ventricular hypertrophy

(RR 1.60, P 0.004). Recently, an additional study was published

from the same population, where the inferior ER patterns were

subgrouped into notched or slurred J-wave patterns and into

ascending or horizontal/descending ST-segments following the

J-wave.48 The risk for arrhythmic death did not differ between

notched and slurred J-wave ER patterns, but the ST segment

morphology distinguished high-risk patterns from benign patterns.

Horizontal/descending ST-segment in the inferior leads was asso-

ciated with a significant risk of arrhythmic death (RR 1.62, 95%

CI 1.192.21), and this pattern combined with a 2-mm J-point ele-

vation further increased the risk (RR 3.37, 95% CI 1.756.51).

Ascending ST-segments after J-waves did not carry an increased

risk (RR 1.01, P NS). Coincidentally, ascending ST-segments

after J-waves was the most prevalent pattern in athletes. Similar

results were described recently from IVF populations by the TelAviv group where horizontal/descending ST-segment after J-point

improved the ability to distinguish IVF patients from matched

controls.49

The second study on ER prevalence and prognosis was con-

ducted in a German population subset of 1945 subjects (age

range 3574 yrs) from the KORA/MONICA cohort.16 The preva-

lence of inferolateral ER (13.1%), with an inferior ER prevalence of

7.6%, was higher than observed in the Finnish study. In the German

study, the risk of cardiac death was increased among inferior ER

carriers and was strongly associated with male gender and

younger age at the time of the ECG recording. Similar to the

Finnish data, males with inferior ER in the age group of 3554years had over four-fold risk of cardiac death.

The third general population study of prevalence and incidence

of ER was conducted in the Nagasaki area in Japan in a population

of atomic bomb survivors.17 Subjects in this study had biennial

physical examination including ECG during the total follow-up

period of 46 years. Interestingly, incident ER findings during the

follow-up were found in 779 subjects and stable ER in 650 sub-

jects resulting in a total prevalence of 29.3%. The mortality rates

in this study were also surprising. Subjects with ER did not have

increased risk of all-cause mortality or cardiac death, but the risk

of sudden unexpected death was significantly elevated among ER

subjects, as in studies conducted in Western populations.

Another recent study explored the prognostic significance of

ER among chronic coronary disease patients with ICD.50 In this

casecontrol study, the prevalence of inferior ER was significantly

higher among patients who had appropriate ICD therapy for

ventricular arrhythmias than in patients who were arrhythmia

free (28 vs. 8%, P 0.011) even after adjustment for LV ejection

fraction.

One noteworthy phenomenon is the considerable age depend-ency of ER prevalence among males. Early repolarization is overre-

presented among young males compared with females, but the

higher prevalence in males declines rapidly during middle age.

This suggests a potential influence of testosterone as a modifier

of J-wave/ER expression, an association also observed in the

Brugada syndrome.45,51 This male preponderance as a function

of age is primarily attributable to the frequency of the benign

ascending ST-segment pattern of ER.

Benign patterns of J-point

elevation and J-waves/earlyrepolarization

The prognostic significance of ER was first comprehensively

studied in a general population, in which subjects with J-point ele-

vation in any lead, including anterior leads, was the only inclusion

criterion.27 The presence of J-waves was not required. In this

study, ER was not found to be associated with increased mortality

risk. Thus, it appears that J-point elevation or ST-segment elevation

itself is not a mortality risk factor in the absence of notching and/or

slurring of the terminal portion of QRS or the formation of appar-

ent J-waves. Among the inferolateral ER pattern carriers, ascending

ST-segment was not associated with increased mortality risk, as

mentioned before.48 Similarly in Brugada syndrome ECGs, the

pattern of the ST-segment morphology plays a role in risk assess-

ment. Several general population-based studies have shown that

Type II or III Brugada ECG finding in a routine ECG screening in

otherwise healthy individual without personal or family history of

SCD or life-threatening arrhythmias is a benign finding.26

J-point elevations and J-waves/earlyrepolarization in athletes

These patterns have been observed in routine ECG recordings

from asymptomatic athletes for many years and have been consid-ered to be normal variants. It is still generally accepted that the

most are indeed benign. However, the association of inferior ER

with SCD has been recently described in an athlete sudden

cardiac arrest (SCA) population from Italy.52 Inferior ER preva-

lence was significantly higher in athlete SCA population compared

with control athlete population (14.3 vs. 2.1%, P 0.017). In the

same study, ST-elevation absence after the J-point elevation was

overrepresented in the SCA victims. Similar findings regarding

ST-segment morphology were found in the Finnish study and in

the study among IVF patients by the Tel Aviv group et al.48,49 In

a recent study of ER among athletes, many distinctive characteris-

tics between inferior and lateral ER carriers were observed. The

Clinical significance of variants of J-points and J-waves Page 3 of 6


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overall prevalence of ER (30%) was strikingly high, as was as the

prevalence of inferior ER (20%) in the mixed ethnicity athlete

population from South Florida.6 The left ventricular hypertrophy

(LVH) pattern determined by SokolowLyon voltage criteria was

present in 25% of inferior ER carriers, but was even more frequent

among lateral ER carriers (40%). Interestingly, African-American

athletes did not have higher prevalence of inferior ER, but did

have significantly more lateral ER. The proposed benign ascendingST-segment morphology with the ER pattern was overrepresented

among the athletes with only 4% overall prevalence of horizontal/

descending ST-segment. The majority (88%) of the ER patterns

with horizontal/descending were detected in inferior leads.36 In

this study during the 10 years of preparticipation ECG screening,

no SCDs or symptomatic ventricular arrhythmias occurred.

Another recent study demonstrated similar findings of ER preva-

lence in another athlete population and also showed that

the prevalence of ER increases during peak training season.53

The increased ER occurrence was independent of echocardio-

graphic findings related to athletes heart, i.e. increased LV

remodelling.

Discussion

Although there are many common features among the various

patterns of J-point elevations and J-waves on electrocardiograms

that demonstrate ER, the associated clinical risks vary from

common benign incidental findings on routine ECGsto patterns sug-

gesting an increased risk of SCD, as in Type I Brugada ECG patterns

and hypothermia-induced J-wave changes. The precise incidence of,

or propensity to, J-point elevation and J-wave generation among a

normal general population, might be even more common than

thought, expressing variably, associated with both specific circum-

stances or occurring in an apparently random fashion. Forexample, J-waves are very common during hypothermia and

during very high vagal tone in some circumstances (e.g. trained ath-

letes), and less commonduring routine ECGscreening. TheBrugada

syndrome and inferolateral ER share the same pathophysiological

characteristics of temporal variation in the ECG pattern, normaliza-

tion during adrenergic states and quinidine administration, and male

predominance (Table1).

In addition to hypothermia, there are at least three different clin-

ically relevant J-wave/ER patterns reported to date. In the Brugada

syndrome, the influence of the ECG pattern is relatively straight-

forward: high penetrance, familial inheritance pattern, and high

risk associated with the Type I pattern. It is noteworthy thatJ-point elevations are observed in all three types of Brugada

ECG patterns, the differences between the three being in the

J-waves and ST T wave patterns. The second association, infero-

lateral patterns associated with IVF, seems to be an entity that

resembles the Brugada syndrome, with similar risk magnitude

and clinical features. Finally, the inferolateral ER pattern associated

with increased mortality risk in the general population may be a

risk modifier interacting with other mechanisms of transient

peaks of risk, such as ischaemia or other proarrhythmic events.

As the KaplanMeier curves of inferior ER show in the Finnish

study,12 the temporal incidence of sudden arrhythmic death

increased around the same age (5070 years) as the mean age

of SCD among coronary disease patients, thus suggesting that

this more prevalent modality of inferolateral ER may indeed

serve a modifying role during acute coronary events. All of the

population-based studies suggesting the adverse prognostic value

of ER focused on the presence of specific J-waves, not only the

presence of consistent, ascending ST-segment elevation.1517

Also in the case control studies only the presence of J-waves

have distinguished patients with idiopathic VF from controls,

whereas ST-segment elevation have not provided any prognostic

value. Therefore, it seems legitimate to draw conclusions that itis mainly the presence of J-waves in ER patients that result in an

increased risk of arrhythmic events. Additionally, the recent cat-

egorization of ascending ST-segment after J-waves in general popu-

lation as a benign trait supports this hypothesis.48

Although clinicians must be aware of the arrhythmogenic poten-

tial of ER, there are emerging data on the ST-segment morphology

that relieve some of the pressure on clinical interpretation of the

relevance of incidental ER patterns created by the publications

from Haissaguerre et al.11 and Tikkanen et al.15 There is also in-

creasing data to suggest that lateral ER may be a benign

finding.1517 In athletes, lateral ER is associated with LVH voltage

and African-American ethnicity, suggesting a normal physiologicvariant to the pattern. Inferior ER amongst athletes lacked such

correlation.6

In a recent commentary, the problem of confusing terminology

was explored.19 Although the terminology surrounding the terms

of J-point elevation and J-waves, vs. ER, can be misleading in

many ways, part of our intent in this review and discussion is to

clarify the language and its implications, blending historical con-

cepts with contemporary observations. The specific patterns that

have been explored in recent years have been shown to convey

risk for arrhythmic death in two separate general population-based

studies. Therefore, regardless of the terminology, this variant has

the potential for arrhythmia prediction that can be seen in an

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Table 1 Common features of early repolarization

and Brugada syndrome

Early repolarization Brugada syndrome

Average age of first event 35

years

Average age of first event 3040

years

Male predominance: 75% Male predominance: 80%Temporal variation in the

expression of the ECG

pattern

Temporal variation in the

expression of the ECG pattern

Vagally mediated accentuation

of ECG pattern

Vagally mediated accentuation of

ECG pattern

Pattern with ascending

ST-segment after J-point:

lower risk

Pattern with ascending ST-segment

after J-point, i.e. Type II and III

ECG: lower risk

Normalization during quinidine

exposure

Normalization during quinidine

exposure

Modified from Myerburg RJ, Castellanos A. Early repolarization and sudden cardiac

arrest: theme or variation on a theme? Nat Clin Pract Cardiovasc Med. 2008;5:760

761.&

Nature Publishing Group.



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