Early Repolarization - UpToDate

3/5/2020 Early repolarization - UpToDate
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Early repolarization
Authors: Andrew Krahn, MD, Manoj Obeyesekere, MBBS
Section Editor: Mark S Link, MD
Deputy Editor: Brian C Downey, MD, FACC
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2020. | This topic last updated: Nov 28, 2018.
INTRODUCTION
The term early repolarization (ER), also known as "J-waves" or "J-point elevation," has long been
used to characterize a QRS-T variant on the electrocardiogram (ECG). Most literature defines ER as
being present on the ECG when there is J-point elevation of ≥0.1 mV in two adjacent leads with either
a slurred or notched morphology. Historically, ER has been considered a marker of good health
because it is more prevalent in athletes, younger persons, and at slower heart rates. However, some
more contemporary reports suggest an association between ER and an increased risk for arrhythmic
death and idiopathic ventricular fibrillation (VF).
While some level of increased risk of sudden cardiac death has been reported in persons with ER, the
relatively high prevalence of the ER pattern in the general population (5 to 13 percent) in comparison
with the incidence of idiopathic VF (approximately 10 cases per 100,000 population) means that the
ER pattern will nearly always be an incidental ECG finding with no clinical implications. However, a
primary arrhythmic disorder such as idiopathic VF due to ER is far more likely when associated with
syncope or resuscitated sudden cardiac death in the absence of other etiologies. (See 'ER syndrome'
below.)
This topic will review the genetics, prevalence, clinical manifestations, and diagnosis of ER and will
present an approach to the management of patients with ER and idiopathic VF.
DEFINITION
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The definition of ER on an ECG is based on well-defined ECG findings (table 1) [1]. Although the
2013 HRS/EHRA/APHRS presented a definition (table 1), the 2016 AHA Scientific Statement [2]
highlights the lack of agreement across published studies pertaining to definition. A 2015 consensus
document suggested reporting more detailed amplitudes of the J-wave including amplitudes
corresponding to J-wave onset (Jo), J-wave peak (Jp), and J-wave termination (Jt), as well as
durations D1 (Jo to Jp) and D2 (Jp to Jt), in relation to an end-QRS notch, and of Jp and Jt, as well as
D2, in relation to an end-QRS slur [3]. The majority of publications at the present time merely adopt
the amplitude of Jp as the reference point for measuring J-point elevation. The ST-segment should be
regarded as horizontal or downward sloping if the amplitude of the ST-segment 100 milliseconds after
Jt (interval M) is less than or equal to the amplitude at Jt. The ST-segment should be regarded as
upward sloping if the amplitude of the ST-segment 100 milliseconds after Jt (interval M) is greater
than the amplitude at Jt. However, duration measurements K, L, and M, each 100 milliseconds, from
Jo, Jp and Jt could be used in the measurement of the ST slope in the presence of a notch or
duration measurements L or M, each 100 milliseconds, used in the presence of an end-QRS slur with
onset from Jp or Jt to measure slope, respectively (figure 1).
ECG findings — On the ECG, ER is defined as either:
● A sharp well-defined positive deflection or notch immediately following a positive QRS complex at
the onset of the ST segment.
● The presence of slurring at the terminal part of the QRS complex (since the J-wave or J-point
elevation may be hidden in the terminal part of the QRS complex, resulting in the slurring of the
terminal QRS complex) (waveform 1).
Most literature defines ER as being present on the ECG when there is J-point elevation of ≥0.1 mV in
two adjacent leads with either a slurred or notched morphology [2,4,5]. The AHA scientific statement
proposed the use of ER qualified with descriptive terms such as with ST-segment elevation, the
magnitude, ER with terminal slur/notch and also noting the distribution on the ECG and any
concomitant ECG findings (eg, J-wave augmentation or short coupled ventricular ectopy).
ECG classification — Based on data associating arrhythmic risk with spatial distribution of ER, a
classification scheme has been proposed [6,7] (see 'Prognosis of ER pattern' below):
● Type 1 is associated with ER in the lateral precordial leads. This form is common among healthy
male athletes and is thought to be largely benign.
● Type 2 is associated with ER in the inferior or inferolateral leads and is associated with a
moderate level of risk.

● Type 3 is associated with ER globally in the inferior, lateral, and right precordial leads, and
appears to be associated with the highest relative risk, though the absolute risk of sudden death
remains small [8].
● Type 4, or Brugada syndrome, is marked by J-wave/point elevation in the right precordial leads.
(See "Brugada syndrome: Clinical presentation, diagnosis, and evaluation".)
While this classification system would seem to simplify categorization of the ECG patterns, it has
been criticized due to the controversial presumed common pathophysiological substrate across the
four types [9,10].
Concealed ER — The ER pattern is not always identified on routine ECG due to the intermittent
nature of ER [11]. As an example, among 542 persons with baseline ER who underwent repeat ECG
examination five years later, ER (≥0.1 mV) was not observed in approximately 20 percent [5]. No
systematic evaluation has been undertaken reporting the prevalence of concealed ER in the general
population, and the clinical importance, if any, of concealed ER remains unclear.
ER pattern versus ER syndrome — As noted above, ER is an ECG finding. Two terms,

distinguished by the presence or absence of symptomatic arrhythmias, have been used to describe
patients with this ECG finding:
● The ER pattern describes the patient with appropriate ECG findings in the absence of
symptomatic arrhythmias.
● The ER syndrome applies to the patient with both appropriate ECG findings and symptomatic
arrhythmias.
Persons with either the ER pattern or ER syndrome can have identical findings on surface ECG.
However, the mere presence of ER pattern on ECG should not lead to a classification of ER
syndrome in the absence of symptoms or documented ventricular fibrillation (VF) [12].
Rarely, ER may be associated with the primary arrhythmic disorder idiopathic VF in the absence of
structural heart disease [4,11]. Given the prevalence of ER pattern in the general population and the
exceedingly low incidence of idiopathic VF, the diagnosis of idiopathic VF due to malignant ER is a
diagnosis of exclusion. (See 'ER syndrome' below.)
PREVALENCE OF ER AND INCIDENCE OF IDIOPATHIC VF
Prevalence — Several population studies have estimated that the prevalence of ER ranges from 5 to
18 percent of persons, with higher prevalence in younger patients [5,13-15].

● In a study of 10,864 middle-aged Finnish subjects (52 percent males, mean age 44±8 years), the
prevalence of ER was 5.8 percent (3.5 percent in inferior leads and 2.4 percent in the lateral
leads, and in both in 0.1 percent) [5].
● In a population based case-cohort study of individuals of central-European descent (n = 6213,

age range 35 to 74 years), the prevalence of ER was 13.1 percent (4.4 percent in the antero-
lateral leads and 7.6 percent in the inferior leads, 1 percent in both) [14]. (See 'Prognosis of ER
pattern' below.)
● In the CARDIA (Coronary Artery Risk Development in Young Adults) study, 5069 participants
(mean age 25 years, 45 percent male, 52 percent black), 941 persons (18.6 percent) had ER on
baseline ECG [16]. After 20 years, there was marked (50 percent) loss to follow-up; however,
only 119 of 2505 persons (4.8 percent) of the remaining participants still had evidence of ER.
Arrhythmic risk — The perception that ER was a benign finding devoid of clinical significance
changed as case reports, case-control studies, and population studies established a link between the
presence of ER and an increased risk for arrhythmic death and in particular idiopathic ventricular
fibrillation (VF) [4,5,8,13-15,17-23].
● Large population studies have shown the presence of ER in the inferior leads on surface ECG is
associated with an increased risk for death from cardiac causes as well as all-cause mortality
[5,15,17].
● In one case-control study which compared 206 subjects with idiopathic VF with 412 healthy
control subjects, ER was more prevalent in subjects with idiopathic VF (31 versus 5 percent), and
ER was greater in magnitude in case subjects than in control subjects (J-point elevation, 2.0
versus 1.2 mm) [4]. Patients with idiopathic VF who had ER were also more likely to experience
syncope or cardiac arrest during sleep than were those without ER. During a mean follow-up of
61±50 months, ICD monitoring showed a higher incidence of recurrent VF in case subjects with
ER than in those without (hazard ratio 2.1, 95% CI 1.2-3.5).
Even though ER is fairly common in the general population, idiopathic VF is rare. In one report, which
estimated the incidence of idiopathic VF, the estimated risk of developing idiopathic VF in an
individual younger than 45 years was 3 in 100,000 [13,24]. The risk increased to 11 in 100,000 when J
waves (waveform 2) were present. Although ER increased the relative risk of sudden cardiac death
(SCD), the absolute risk was very low. In a meta-analysis, the relative risk of arrhythmic death in
persons with the ER pattern was 1.70 (95% CI 1.19-2.42), and the estimated absolute risk for
arrhythmic death was 70 per 100,000 person-years [25]. Therefore the incidental identification of ER
should not be interpreted as a high-risk marker for arrhythmic death due to the relatively low odds of
SCD based on ER alone.

Athletes with early repolarization — It remains controversial whether athletes have an increased
prevalence of ER compared with the general population and an increased risk for arrhythmic death
[26-29].
● The prevalence of J-point elevation among 121 young athletes was reported at 22 percent, a
prevalence rate higher than seen in the general population [13]. However, higher ER prevalence
rates ranging as high as 44 percent have also been reported in athletes [30,31].
The reported higher prevalence of ER in athletes likely is related to the physiological balance in
autonomic tone favoring the parasympathetic tone and its regulation of the action potential [32].
In athletes, a correlation between J point elevation and interventricular septal thickness has been
reported and may suggest a mechanistic role of exercise-induced hypertrophy as the basis for J
point elevation [33].
● Another case control study reported that ER was four times more prevalent among athletes with
a history of cardiac arrest (n = 21) than among healthy athletes (n = 365) [29]. However, in this
study, the prevalence of ER in the control group of athletes was substantially lower (7.9 percent)
in comparison with other studies. The presence of ER increased the probability of arrhythmic
death from approximately 2 per million to 3.5 per million in this population of competitive athletes
[29].
● In a cohort of 704 elite Italian athletes without apparent cardiovascular disease, the ER pattern
was present in 102 persons (14 percent) [34]. During a mean follow-up of six years, none of the
athletes with ER developed ventricular arrhythmia or sudden cardiac death.
● In young healthy athletes from Finland (n = 62) and the United States (n = 503), an ascending ST
segment was the common form of ER, which has not been associated with an increased
arrhythmic risk [30]. Amongst athletes with ER, all but one of the Finnish (96 percent) and 85
percent of US athletes had an ascending ST variant after ER. (See 'Prognosis of ER pattern'
below.)
Notably, the association of ER with arrhythmic risk is typically at rest or during sleep and not during
physical activity when J-point elevation is typically markedly reduced or eliminated.
PROGNOSIS OF ER PATTERN
Certain ECG characteristics may distinguish the benign ER pattern from patterns associated with a
higher arrhythmic risk. Additionally, ER pattern may be modified by physiological variables with
subsequent effect on prognosis. The coexistence of ER with other cardiac pathologies also likely
influences prognosis/arrhythmic risk. Reports suggest that ER should be viewed as an adjunctive
prognostic variable in the presence of other cardiac pathologies (ie, ER may incrementally worsen the
prognosis of other more common conditions such as ischemic heart disease).
Prognosis in the general population — While the ER pattern is associated with an increased
relative risk of adverse events, the prognostic implications described in this section should be viewed
in the context of the overall very low risk of SCD in those with this asymptomatic ECG finding. Thus,
even with a twofold increase in relative risk of SCD, the absolute risk remains exceedingly low.
Additionally, in spite of the data discussed in this section, there is no current risk stratification strategy
for asymptomatic patients with ER pattern in the general population and within families with ER
pattern that would allow for the identification of higher risk individuals with the ER pattern who might
be candidates for treatment. (See 'Prevalence' above and 'Arrhythmic risk' above and 'Treatment of
ER pattern' below.)
The presence of the ER pattern has generally been associated with adverse outcomes in numerous
cohort and case-control studies, although some studies have suggested no overall impact on mortality
after adjusting for comorbidities including coronary risk factors [4,5,13-15,17,25,35-38].
● In a 2016 meta-analysis of sixteen studies involving 334,524 subjects with follow-up ranging from
6 to 30 years, persons with the ER pattern had a significantly greater risk of sudden cardiac
arrest (relative risk [RR] 2.18, 95% CI 1.29-3.68) as well as cardiac death (RR 1.48, 95% CI 1.06-
2.07) and all-cause mortality (RR 1.21, 95% CI 1.2-1.42) compared with those without the ER
pattern [38].
● However, in a 2015 study in which investigators manually coded the ECGs of a 20,661 person
cohort to identify J-waves and QRS slurs (seen in 4219 persons), and followed them for a median
of 17.5 years, no significant increase in the risk of cardiovascular death was noted in persons
with ER pattern [39].
Prognostic variables — Variables thought to affect prognosis that have been investigated include:
● Distribution and amplitude of ER

● Morphology of the J wave, ST segment, and T wave
● Gender
● Family history
● Slurring versus notching
● Ethnicity
● Association with other cardiac pathology
Distribution and amplitude of early repolarization — The inferior location of ER, in addition to
higher J-point amplitude, have been described as variables associated with increased arrhythmic risk

in both the general population and in patients with idiopathic ventricular fibrillation (VF), although
some conflicting data have been reported in the general population.
● J-point elevation greater than 0.1 mV in the inferior leads in the large Finnish cohort study of
10,864 persons was weakly associated with an increased risk for death from cardiac causes
(adjusted RR 1.28, 95% CI 1.04-1.59) [5]. While observed in only 0.3 percent of the cohort, J-
point elevation greater than 0.2 mV in inferior leads (waveform 3) was associated with a three
times greater risk of death from cardiac causes (adjusted RR 2.98, 95% CI 1.85-4.92) [5]. Similar
findings have been reported in a series of 40 patients with idiopathic VF, in whom the J-point was
nearly two times higher above the baseline than the height seen in controls without VF [40].
● In a population-based case-cohort study of 6213 persons (1945 persons with ECGs) with a mean
follow-up of 19 years, ER was associated with higher cardiac mortality (hazard ratio 1.96, 95% CI
1.05-3.68) [14]. An inferior localization of ER further increased ER-attributable cardiac mortality
(hazard ratio 3.15, 95% CI 1.58-6.28. However, these findings were not replicated in a cohort of
20,661 patients (90.5 percent male) with a median follow-up of 17.5 years, among whom the
findings of ER were not associated with an increased risk of cardiovascular death [39].
● The coexistence of anterior ER was reported as a key predictor of poor outcomes in patients with
inferolateral ERS [41].
Morphology of the J wave, ST segment, and T wave — The morphology of the ST segment
may also determine the risk associated with ER, with a horizontal or downsloping ST segment
following ER portending a higher risk in both the general population and in patients with idiopathic VF
[24,30,36]. However, despite the increased risk of arrhythmia associated with the
horizontal/downsloping ER pattern, the prevalence of this pattern in controls (approximately 3
percent) compared with the exceedingly low incidence of idiopathic VF renders this variable alone
devoid of meaningful test accuracy [24,30]. In one study that compared 92 patients with ER and VF
with 247 control patients with asymptomatic ER pattern, patients with ER and VF had a higher
prevalence of low-amplitude T waves, lower T/R ratio (lead II or V5), and longer QTc interval [42]. The
authors suggested the combination of these parameters with J-wave amplitude and distribution of J
waves may further allow for improved identification of malignant ER.
J-wave duration (interval from Jo and the intersection of the tangent to the J wave with the isoelectric
line >60 milliseconds) and slope/j-angle (angle between an ideal line drawn from Jo perpendicular to
the isoelectric line and the tangent to the J wave >30°) have been reported to identify the malignant
form of ER from the benign form [43].
Gender — Men with ER in the inferior leads may be at greater risk of cardiac mortality than men
without ER or women with or without ER [14].

Family history — Conflicting data exist regarding the prognostic significance of a family history of
sudden cardiac death (SCD) in persons with ER, such that no clear recommendations regarding risk
assessment or treatment can be made [4,44]. Further studies are required to define the risk
contribution of a family history of SCD to the prognosis of a patient with ER. Society guidelines
support a possible role for an ICD in symptomatic family members of ER syndrome patients with a
history of syncope in the presence of ST-segment elevation, or asymptomatic individuals with a high-
risk ER pattern in the presence of a family history of early sudden death. The latter should be
exceptional, in discussion with an expert versed in assessment of familial sudden death syndromes
[45].
Slurring versus notching — Although both slurring and notching type ER are observed and may
exist in the same patient, the prognostic value of one compared with the other has not been clearly
established (waveform 1 and figure 2) [11,15,24,36,39,46].
Ethnicity — Although ER is more common in African Americans, there is no clear attributable risk
associated with ethnicity, and African Americans are not specifically over represented in idiopathic VF
cohorts [47]. The ER pattern itself, however, does appear to be more commonly associated with
African American ancestry [16].
ER modifying risk of underlying cardiac pathology — In the context of structural heart disease
and primary electrical disorders, the ER pattern may be a modifier of the underlying arrhythmic risk
associated with these heterogeneous cardiac conditions, in addition to the rare association with
idiopathic VF (ie, in the absence of other cardiac conditions). As examples:
● Patients with ECG findings of the ER pattern appear to be at an increased risk of VF in the event
of a myocardial infarction/ischemia [48,49]. Abbreviation of the epicardial action potential occurs
during acute myocardial ischemia, with the decrease in the action potential contributing to ST-
segment elevation [50]. (See "Ventricular arrhythmias during acute myocardial infarction:
Incidence, mechanisms, and clinical features", section on 'Ventricular fibrillation'.)
● The ER pattern in the inferior leads has also been demonstrated to be associated with an
increased risk of life-threatening ventricular arrhythmias in patients with chronic coronary artery
disease, after adjustment for left ventricular ejection fraction [51].
● The ER pattern in the inferior leads has been associated with an increased risk of sudden cardiac
death in patients with congestive heart failure [52].
● A high prevalence of the ER pattern in patients with short QT syndrome has been reported [53].
In multivariate models, ER was associated with arrhythmic events in the short QT syndrome
cohort. (See "Short QT syndrome".)

● There is speculation that SQTS and ER syndrome are overlap syndromes since many patients
with ER syndrome have a relatively short QT interval [54].
● The ER pattern has also been demonstrated to be prevalent in patients with arrhythmogenic right
ventricular cardiomyopathy (31 percent), though this retrospective analysis identified no
correlation between ER and clinical findings with regard to history of previous cardiac arrest,
syncope, or arrhythmic events [55]. (See "Arrhythmogenic right ventricular cardiomyopathy:
Anatomy, histology, and clinical manifestations", section on 'Ventricular arrhythmias'.)
● A high prevalence of ER in noncompaction cardiomyopathy patients has been reported,

especially in those who present with malignant ventricular arrhythmias [56].
● ER may also modify the clinical course in long QT syndrome (LQTS), with ER ≥2 mm reported as
an independent predictor of symptom status related to LQTS, along with female sex and QTc
>500 milliseconds [57]. This aligns with previous observations that it is a common risk modifier
and rare primary arrhythmogenic syndrome.
● ER has been demonstrated to modify symptoms in patients with catecholaminergic polymorphic

ventricular tachycardia (CPVT), demonstrating an increased risk of syncope in patients with
CPVT in those with ER compared with those without ER [58].
Among 51 patients with CPVT patients (mean age 36±15 years, 11 males), the ER pattern was
present in 23 (45 percent), and all patients with ER pattern were symptomatic at presentation (23
of 23 patients versus 19 of 28 patients without ER pattern) [58]. Syncope was also more frequent
in patients with ER pattern (18 of 23 patients versus 11 of 28 patients without ER pattern).
In a study involving 22 men with Brugada syndrome and electrical storm (defined as ≥3 episodes
per day of VF) compared with 110 age-matched, control men with Brugada syndrome without
electrical storm, the prevalence and magnitude of ER was higher in patients with than without
electrical storm [59].
Thus, the ER pattern is potentially a marker for increased arrhythmic mortality due to a vulnerable
repolarization substrate when combined across a number of heterogeneous clinical conditions (eg,
short QT syndrome, Brugada syndrome, or ischemic heart disease) in the general population, in
addition to rarely being a primary arrhythmogenic disorder.
Risk stratification with invasive EP studies — Invasive electrophysiologic studies (EPS) do not
appear to improve the risk stratification of patients with the ER syndrome and prior VF arrest [60].
(See 'Chronic treatment of ER syndrome' below and "Invasive diagnostic cardiac electrophysiology
studies".)

GENETIC BASIS AND INHERITANCE OF ER
The genetic basis of ER syndrome continues to be elucidated, with the evidence restricted to either
case reports or preliminary studies that fall short of clearly identifying the genetic basis of ER [61,62].
The reported implicated gene mutations involve the KCNJ8 gene (responsible for the ATP-sensitive
potassium channel Kir6.1 - IKATP current); CACNA1C, CACNB2, CACNA2D1 genes (responsible for
the cardiac L-type calcium channel - ICa.L current); and the SCN5A gene (responsible for the sodium
channel - INa current) (figure 3) [61-65]. First-degree relatives of patients with the ER pattern are more
likely to also demonstrate the pattern, but this weak association has not been associated with clinical
implications [66,67].
Inheritance of ER pattern — The ER pattern may be sporadic or inherited, although first-degree

relatives of a person with the ER pattern appear to have a two to threefold higher likelihood of also
having the ER pattern on ECG [66,67]. While the vast majority of ER is likely sporadic, familial ER
appears to be transmitted in an autosomal dominant fashion [68].
● In one study that evaluated participants in the Framingham Heart Study (n = 3995) and the
Health 2000 Survey (n = 5489), siblings of individuals with ER pattern had increased unadjusted
odds of having ER pattern on their ECG (odds ratio [OR] 2.22, 95% CI 1.01-4.85), suggesting
heritability of the ER pattern in the general population [66].
● In a study of 505 families, individuals for whom at least one parent had the ER pattern had a 2.5-
fold risk for ER pattern [67]. Familial transmission appeared more frequent when the mother was
affected (3.8-fold versus 1.8-fold). Heritability was also higher when ER was in the inferior leads
or had a notched morphology. (See 'Family history' above.)
● In a study of four families affected by ER syndrome with a combined 22 sudden cardiac deaths,
the ER pattern was present in 36 percent of screened family members (61 out of 171), with
transmission in a fashion consistent with autosomal dominant inheritance [68].
Gain of function mutations — Consistent with the reports that IKATP activation can generate an ER
pattern on the surface ECG, several investigators have detected a rare missense mutation, S422L in
KCNJ8, to be associated with ER and idiopathic ventricular fibrillation (VF) [61,63,64].
● The first report on this variant was a case report of a 14-year-old female who experienced
numerous episodes of recurrent idiopathic VF unresponsive to beta-blockers, multiple anti-
arrhythmic medications and verapamil [61]. Recurrences of VF were associated with a marked
accentuation of ER.

● In a study of 87 patients with Brugada syndrome and 14 patients with ER syndrome, one
Brugada syndrome case and one ER syndrome case hosted the identical missense mutation
S422L. Investigators demonstrated that IKATP was increased significantly in the S422L variant
compared with Kir6.1 wild type channels [63].
● A separate study of 204 patients and family members with Brugada syndrome or ER syndrome
also demonstrated a similar gain of function of the Kir6.1 channel (identified in three Brugada
syndrome and one ER syndrome proband) [64].
KCNJ8-S422L is highly conserved across species and was absent in the reference alleles in these
three studies [61,63,64]. This gain of function variant appears to be pathogenic in ER and idiopathic
VF.
Loss of function mutations — Loss of function mutations of the inward sodium channel gene and
cardiac L-type calcium channel gene have also been implicated in patients with ER (CACNA1C,
CACNB2, and CACNA2D1 genes). Two small studies (three and four patients with ER, respectively)
have reported that mutations in these highly conserved residues were associated with ER, suggesting
linkage of these genes with ER [62,65].
The complex genetic basis continues to be explored. Highlighting the complexity, a study indicated
that the c.4297 G>C missense mutation in the SCN5A gene caused a "loss-of-function" of sodium
channels accounting for the ERS in a single case [69]. The reduction in INa density was due to a
decreased number of sodium channels caused by abnormal translation processes. However, the
synonymous T5457C polymorphism on the same allele partially restored the INa density of the mutant
channels by the upregulation of mRNA levels.
A genome-wide association study was not able to reliably identify genetic variants predisposing to ER,
presumably due to insufficient statistical power and phenotype heterogeneity [70]. This does however
suggest that the genetic mechanisms may be multifactorial and that an ER gene or family of genes is
an unlikely outcome of further ascertainment.
MECHANISM OF ER AND IDIOPATHIC VENTRICULAR FIBRILLATION
ER mechanistically demonstrates some similarities to Brugada Syndrome and short QT syndrome

(SQTS). Although the exact mechanism for ER is still unknown and the precise mechanism for ER-
related idiopathic ventricular fibrillation (VF) is also unknown, fundamentally, the purported
mechanisms all reflect an imbalance in the ion channel currents (figure 3 and figure 2) responsible for
the terminal portion of depolarization and the early portion of repolarization. (See "Short QT
syndrome" and "Brugada syndrome: Epidemiology and pathogenesis", section on 'Pathogenesis'.)

There is controversy regarding whether the ER pattern represents repolarization or depolarization.
● In a study of 206 persons with idiopathic VF and the ER pattern, only a minority of cases (11
percent) had late potentials on signal averaged ECG, with a prevalence similar to the control
group who did not have the ER pattern (13 percent), suggesting that ER is not a depolarization
phenomenon (figure 4) [4].
● However, in a smaller study of 22 patients with apparently idiopathic VF who were monitored
using a signal-averaging system to record depolarization markers, repolarization markers, and
autonomic modulation, the incidence of late potentials in persons with VF was significantly higher
in those with the ER pattern (86 percent versus 27 percent in those without ER pattern) [18]. In
contrast, repolarization markers did not differ between the two groups. These investigators
concluded that ER might be more closely associated with a depolarization abnormality and
autonomic modulation than with repolarization.
Efforts are ongoing to more clearly elucidate the mechanism of ER and to determine if it is
fundamentally related to depolarization or repolarization.
CLINICAL MANIFESTATIONS AND DIAGNOSIS
ER pattern — Given the relatively high prevalence of the ER pattern in the general population (5 to
13 percent) in comparison with the incidence of idiopathic ventricular fibrillation (VF) (approximately
10 cases per 100,000 population), the ER pattern is nearly always a benign incidental ECG finding.
There are no specific signs or symptoms attributed to the ER pattern, which is identified through the
use of a standard ECG. In the absence of syncope or sudden cardiac arrest, no additional testing is
required in persons with the ER pattern.
Asking patients to perform the Valsalva maneuver may unmask or accentuate the ER pattern. While
performing this maneuver has been shown to aid in the identification of the ER pattern in high-risk
familial ER, the report of this has not been validated and its applicability to broad populations of
asymptomatic persons has not been evaluated [68]. The Valsalva maneuver is discussed in greater
detail separately. (See "Vagal maneuvers".)
ER syndrome — Patients with ER syndrome typically present with sudden cardiac arrest due to VF.
While patients with ER syndrome may rarely present with syncope, this appears to be unusual, and
syncope has not been shown to be more common in patients with ER pattern [66,71]. However,
another study of 259 patients with neurally-mediated syncope demonstrated head up tilt table-positive
rate to be higher in patients with J-waves compared with patients without J-waves [72]. Such patients
who do present with syncope are likely to display high-risk features of ER and may have a vagal

prodrome. However, most persons with the ER pattern identified on ECG who experience an isolated
episode of syncope, especially syncope which appears non-cardiac in origin, will not be diagnosed
with the ER syndrome in the absence of additional data showing VF.
The diagnosis of ER syndrome is most commonly considered in a survivor of sudden cardiac death
(SCD) with ECG evidence of VF and an apparently structurally normal heart following extensive
testing (table 1). A systematic assessment of the survivors of sudden cardiac death without evidence
of infarction or left ventricular dysfunction is reported to establish a causative diagnosis in the majority
of cases [11,73]. Systematic evaluation includes:
● Cardiac monitoring
● Signal-averaged ECG
● Exercise testing
● Echocardiogram
● Cardiac magnetic resonance imaging
● Evaluation of coronary arteries, typically with invasive angiography
● Intravenous adrenaline and sodium channel blocker challenge
● Targeted genetic testing should also be considered when a phenotype is suggested by the above
evaluation (eg, long QT syndrome, Brugada syndrome, catecholaminergic polymorphic
ventricular tachycardia) (see "Congenital long QT syndrome: Diagnosis" and "Catecholaminergic
polymorphic ventricular tachycardia" and "Brugada syndrome: Clinical presentation, diagnosis,
and evaluation", section on 'Diagnosis')
In patients whose evaluation revealed no identifiable cardiac pathology, idiopathic VF and the ER
syndrome should be considered. A careful review of all available ECGs for evidence of ER is
warranted, particularly around the time of the cardiac arrest [11]. ECGs often show variable and at
times absent ER, so thorough review is important. VF storms in the idiopathic VF patients attributed to
ER syndrome were highly associated with J wave augmentation prior to the VF onset [74].
ER syndrome causing VF may be diagnosed when:
● Other etiologies have been systematically excluded

● When J-point elevation is augmented immediately preceding VF
ER syndrome causing VF is probable when:
● Other etiologies have been systematically excluded

● ER pattern exists or increased parasympathetic tone provokes ER
● Cardiac arrest occurs at rest or during sleep

These patients may also display a high-risk ER pattern (J-point elevation >2 mm in the inferior or
inferolateral leads or globally and/or horizontal or down sloping ST segment. (See 'Prognosis of ER
pattern' above.)
The ER pattern is not always identified on routine ECG due to the intermittent nature of ER.
Bradycardia dependent and vagally dependent augmentation of ER has been reported [75]. However,
no provocative test, such as pharmacologic augmentation of parasympathetic tone, is currently
available and validated in this setting. Although the utility has not been studied systematically, tilt table
testing may be of assistance to establish if vagal stimulation is associated with VF/syncope and/or if
high-risk ER features are provoked. However, the diagnostic accuracy of this approach is unknown,
and vasovagal syncope is far more common in comparison with VF due to ER syndrome. (See
'Prognostic variables' above and "Upright tilt table testing in the evaluation of syncope".)
DIFFERENTIAL DIAGNOSIS
ER versus Brugada syndrome — Some individuals with Brugada syndrome (ECG findings of a
pseudo-right bundle branch block and persistent ST segment elevation in leads V1 to V2 associated
with SCD or sustained ventricular arrhythmia) also have ER (approximately 12 percent) as variants in
genes encoding the L-type calcium channel, ATP-sensitive potassium channel, and sodium channels
have been associated with both of these conditions [62,63,65,76-79]. Additionally, some ECG
characteristics of ER resemble features of the Brugada ECG, including J waves, pause and
bradycardia dependent accentuation, the dynamic nature of the ECG manifestations, short-coupled
extra-systole-induced polymorphic ventricular tachycardia/ventricular fibrillation, and suppression of
the ECG features and arrhythmia with isoproterenol and quinidine [8,80].
However, the Brugada ECG feature of provocation by sodium channel blocker is not observed in ER
[81]. In fact, sodium channel blockers in most patients with ER attenuate the J-point, whereas the J-
point is augmented by sodium-channel blockers in the right precordial leads in patients with a
Brugada ECG. Additionally, in a study comparing the ECG features of 61 athletes with ER with 92
patients with the Brugada syndrome, patients with the Brugada syndrome had significantly greater ST
segment elevation at the J-point (3.3 versus 1.5 mm), with the Brugada syndrome patients universally
showing downsloping ST segments (compared with only 2 of 61 athletes) [82]. Furthermore, a
positive signal-averaged ECG and structural abnormalities in the right ventricular outflow tract are not
consistently observed and have not been reported in patients with ER, respectively [83-85]. (See
"Brugada syndrome: Clinical presentation, diagnosis, and evaluation".)
ER versus acute pericarditis — As is seen in ER, there is J-point elevation with resultant ST
segment elevation in patients with acute pericarditis. Symptom presentation is markedly different in

the two conditions. Unlike ER, most patients with acute pericarditis have ST elevations diffusely in
most or all limb and precordial leads. Additionally, patients with acute pericarditis often have deviation
of the PR segment, which is not present in ER. (See "Acute pericarditis: Clinical presentation and
diagnostic evaluation", section on 'Electrocardiogram'.)
ER versus acute myocardial injury — While patients with acute myocardial injury due to ST
elevation myocardial infarction (STEMI) can initially have elevation of the J-point with concave ST
segment elevation, the ST segment elevation typically becomes more pronounced and convex
(rounded upward) as the infarction persists. However, the primary distinguishing factor between ER
and acute myocardial injury is the presence of clinical symptoms such as chest pain or dyspnea. The
distinction between the ECG findings of ER and acute MI are discussed in greater detail elsewhere.
(See "Electrocardiogram in the diagnosis of myocardial ischemia and infarction", section on 'Early
repolarization'.)
TREATMENT
Treatment of ER pattern — As discussed above, the ER pattern is nearly always a benign incidental
ECG finding, with no specific signs or symptoms attributed to it. In addition, there is no current risk
stratification strategy for asymptomatic patients with ER pattern in the general population and within
families with ER pattern that would allow for the identification of higher risk individuals with the ER
pattern who might be candidates for treatment. The 2017 AHA/ACC/HRS guideline for management
of patients with ventricular arrhythmias and the prevention of sudden cardiac death recommends
observation with no treatment, whereas the 2015 European Society of Cardiology guidelines on the
treatment of ventricular arrhythmias and prevention of sudden cardiac death found that there was
"insufficient evidence" to make a recommendation on the management of the ER pattern without
associated symptomatic ventricular arrhythmias [12,86].
As such, for patients with the incidental finding of the ER pattern on their ECG, we recommend
observation without therapy (table 1).
Treatment of ER syndrome with idiopathic VF — Among survivors of SCD due to idiopathic

ventricular fibrillation (VF), the reported rate of recurrent VF ranges between 22 and 37 percent at two
to four years [87-89]. Because these patients have no structural heart disease, they have an excellent
prognosis for long-term survival if VF is treated (algorithm 1). As a result, such patients are best
treated with an implantable cardioverter-defibrillator (ICD) [12,86-91]. (See "Secondary prevention of
sudden cardiac death in heart failure and cardiomyopathy".)
Acute treatment of ER syndrome with VF storm — For patients with ER syndrome and ongoing
acute VF requiring frequent defibrillation, we suggest intravenous isoproterenol (table 1). In a
multicenter observational cohort study of 122 patients (90 males, mean age 37±12 years) with ER in
the inferolateral leads and more than three episodes of idiopathic VF (including those with electrical
storm), isoproterenol was effective for the acute suppression of VF, immediately suppressing electrical
storms in seven of seven patients [92]. Theophylline has been described in a case report as
eliminating ongoing malignant ventricular arrhythmias in spite of treatment with quinidine and high-
rate ventricular pacing [93]. High-dose flecainide has also been reported to be effective at
suppressing VF in ERS in a case report [94].
In an experimental canine model of ER, quinidine, cilostazol, and milrinone, each administered
individually, have been shown to suppress hypothermia-induced ventricular arrhythmias [95]. There
are insufficient human data to comment on the possible therapeutic use of these drugs to treat acute
ventricular arrhythmias.
Chronic treatment of ER syndrome — Chronic treatment of the ER syndrome should include an

implantable cardioverter-defibrillator (ICD) for rapid treatment of any recurrent VF (table 1) [12,45].
Patients with frequent recurrent episodes of VF resulting in ICD shocks may require suppressive
therapy with an antiarrhythmic drug and, rarely, ablation of a stereotypic initiating premature
ventricular complex/contraction (PVC; also referred to a premature ventricular beats or premature
ventricular depolarizations).
● For patients with ER syndrome with prior resuscitated sudden cardiac death (SCD) due to VF, we
recommend implantation of an implantable cardioverter-defibrillator (ICD) for secondary
prevention of SCD. ICD therapy is highly effective in terminating ventricular arrhythmias in nearly
all cases. The potential benefit of the ICD in patients with idiopathic VF was illustrated in a report
of 28 survivors of VF with minimal or no structural heart abnormalities, in whom there were 36
ICD shocks in 16 patients but no cardiac deaths over an average follow-up of 30 months [91].
● Antiarrhythmic drug therapy is a therapeutic option for patients with recurrent VF following ICD
implantation. For patients with ER syndrome and recurrent VF, we suggest the use of quinidine, a
class IA antiarrhythmic drug, for chronic suppressive therapy. Class IA antiarrhythmic drugs have
been shown to prevent reinduction of polymorphic ventricular arrhythmias both during
electrophysiologic (EP) study and in long-term follow-up in patients with idiopathic VF [87,96].
Twenty-three patients with idiopathic VF and inducible arrhythmia at EP study were treated with
the class IA drug that was effective during EP study (usually quinidine); during a mean follow-up
of 9.1 years, no patient died or had a sustained ventricular arrhythmia [96]. The target dose
should be no less than 1000 mg per day, with a common target of 1200 to 1600 mg of quinidine
sulfate divided in four doses. (See "Pharmacologic therapy in survivors of sudden cardiac
arrest".)

● In a case report of a patient with recurrent VF storm in spite of 1200 mg per day of quinidine and
subsequently bisoprolol, cilostazol, and amiodarone, flecainide markedly reduced the number of
VF episodes [94].
● For patients with ER syndrome and prior idiopathic VF but no documented recurrent arrhythmias,
we do not suggest chronic suppressive treatment with an antiarrhythmic drug as the frequency of
recurrent VF attributed to ER syndrome is highly variable and not readily predicted.
● In an in vitro pharmacological model, the phosphodiesterase III inhibitors cilostazol or milrinone

have been demonstrated to diminish ER manifestations and prevent the hypothermia-induced
phase 2 reentry and ventricular tachycardia (VT)/VF [95]. Quinidine also demonstrated similar
effects in this model. In coronary-perfused canine left ventricular wedge preparations, PDE-3
inhibitors (cilostazol and milrinone or isoproterenol) have been demonstrated to exert an
ameliorative effect on induced arrhythmias by producing an inward shift in the balance of current
during the early phases of the epicardial accessory pathway via inhibition of Ito as well as
augmentation of ICa, thus reversing the repolarization defects underlying the development of
phase 2 reentry and VT/VF [97].
Patients with ER syndrome who participate in competitive athletics require further evaluation and
appropriate precautions prior to returning to competition [98].
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around
the world are provided separately. (See "Society guideline links: Inherited arrhythmia syndromes" and
"Society guideline links: Ventricular arrhythmias".)
SUMMARY AND RECOMMENDATIONS
● Early repolarization (ER) is defined as either a sharp well-defined positive deflection or notch
immediately following a positive QRS complex at the onset of the ST segment, or the presence of
slurring at the terminal part of the QRS complex (since the J-wave or J-point elevation may be
hidden in the terminal part of the QRS complex, resulting in the slurring of the terminal QRS
complex) (waveform 1). Most literature defines ER as being present on the electrocardiogram
(ECG) when there is J-point elevation of ≥0.1 mV in two adjacent leads with either a slurred or
notched morphology. (See 'ECG findings' above.)

● ER is an ECG finding. Two terms, distinguished by the presence or absence of symptomatic

arrhythmias, have been used to describe patients with this ECG finding (see 'ER pattern versus
ER syndrome' above):
• The ER pattern describes the patient with appropriate ECG findings in the absence of
• The ER syndrome applies to the patient with both appropriate ECG findings and
The mere presence of ER pattern on ECG should not lead to a classification of ER syndrome in
the absence of symptoms or documented ventricular fibrillation (VF).
● Several population studies have estimated that the prevalence of ER ranges from 5 to 13 percent
of persons. The perception that ER was a benign finding devoid of clinical significance has
changed, with numerous studies suggesting a two- to threefold increased risk of death in those
with ER versus those without ER. While ER appears to increase one's risk of sudden cardiac
death (SCD), the absolute risk of SCD remains exceedingly low in otherwise healthy people.
(See 'Prevalence' above and 'Arrhythmic risk' above.)
● The genetic basis of ER continues to be elucidated, with the evidence restricted to either case
reports or preliminary studies that fall short of clearly identifying the genetic basis of ER. (See
'Genetic basis and inheritance of ER' above.)
● The purported mechanisms of ER and idiopathic VF all reflect an imbalance in the ion channel
currents responsible for the terminal portion of depolarization and the early portion of
repolarization. (See 'Mechanism of ER and idiopathic ventricular fibrillation' above.)
● Given its relatively high prevalence in the general population in comparison with the incidence of
idiopathic VF, the ER pattern is almost always an incidental ECG finding. The diagnosis of ER
syndrome, however, should be considered in a survivor of sudden cardiac death (SCD) with ECG
evidence of ER and VF and an apparently structurally normal heart following extensive testing.
(See 'ER pattern' above and 'ER syndrome' above.)
● For patients with the incidental finding of the ER pattern on their ECG, we recommend
observation without therapy (Grade 1A). (See 'Treatment of ER pattern' above.)
● For patients with ER and ongoing acute VF (VF storm) requiring frequent defibrillation, we
suggest intravenous isoproterenol (Grade 2C). (See 'Acute treatment of ER syndrome with VF
storm' above.)

● For patients with ER syndrome with prior resuscitated SCD due to VF, we recommend
implantation of an implantable cardioverter-defibrillator (ICD) for secondary prevention of SCD
(Grade 1A). (See 'Chronic treatment of ER syndrome' above.)
● For patients with ER syndrome and recurrent VF, we suggest the use of quinidine, a class IA
antiarrhythmic drug, for chronic suppressive therapy (Grade 2C). (See 'Chronic treatment of ER
syndrome' above.)
● Patients with ER syndrome who have had an ICD placed but who have had no documented
recurrent arrhythmias do not require chronic antiarrhythmic drug treatment. (See 'Chronic
treatment of ER syndrome' above.)
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standardized clinical evaluation. Consensus Statement of the Joint Steering Committees of the
Unexplained Cardiac Arrest Registry of Europe and of the Idiopathic Ventricular Fibrillation
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significant structural heart disease: a multicenter experience with implantable cardioverter-
defibrillator therapy. J Am Coll Cardiol 1993; 21:1406.

92. Haïssaguerre M, Sacher F, Nogami A, et al. Characteristics of recurrent ventricular fibrillation

associated with inferolateral early repolarization role of drug therapy. J Am Coll Cardiol 2009;
53:612.
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arrhythmia associated with early repolarization. Pacing Clin Electrophysiol 2018; 41:444.
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patient with early repolarization syndrome. Heart Rhythm 2016; 13:1724.
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ventricular tachycardia/ventricular fibrillation in the setting of early repolarization and the
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7:134.
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effects of cilostazol, milrinone, and isoproterenol to suppress arrhythmogenesis in an
experimental model of early repolarization syndrome. Heart Rhythm 2016; 13:1326.
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American College of Cardiology. J Am Coll Cardiol 2015; 66:2424.
Topic 83199 Version 24.0

GRAPHICS
Expert consensus recommendations regarding early repolarization from the

HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management
of Patients with Inherited Primary Arrhythmia Syndromes
Expert consensus statement on diagnosis

1. ER syndrome is diagnosed in the presence of J-point elevation ≥1 mm in ≥2 contiguous inferior and/or lateral
leads of a standard 12-lead ECG in a patient resuscitated from otherwise unexplained VF/polymorphic VT.
2. ER syndrome can be diagnosed in an SCD victim with a negative autopsy and medical chart review with a previous
ECG demonstrating J-point elevation ≥1 mm in ≥2 contiguous inferior and/or lateral leads of a standard 12-lead ECG.
3. ER pattern can be diagnosed in the presence of J-point elevation ≥1 mm in ≥2 contiguous inferior and/or lateral
leads of a standard 12-lead ECG.
Expert consensus statement on therapeutic intervention

CLASS I
1. ICD implantation is recommended in patients with a diagnosis of ER syndrome who have survived a cardiac arrest.
CLASS IIa
2. Isoproterenol infusion can be useful in suppressing electrical storms in patients with a diagnosis of ER syndrome.
3. Quinidine in addition to an ICD can be useful for secondary prevention of VF in patients with a diagnosis of ER
syndrome.
CLASS IIb
4. ICD implantation may be considered in symptomatic family members of ER syndrome patients with a history of
syncope in the presence of ST-segment elevation >1 mm in ≥2 inferior or lateral leads.
5. ICD implantation may be considered in asymptomatic individuals who demonstrate a high-risk ER ECG pattern
(high J-wave amplitude, horizontal/descending ST segment) in the presence of a strong family history of juvenile
unexplained sudden death with or without a pathogenic mutation.
CLASS III
6. ICD implantation is not recommended in asymptomatic patients with an isolated ER ECG pattern.
ECG: electrocardiogram; VF: ventricular fibrillation; VT: ventricular tachycardia; ER: early repolarization; SCD: sudden cardiac
death; ICD: implantable cardioverter defibrillator.
Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of
Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013; 10:1932. Table used with the permission of
Elsevier Inc. All rights reserved.
Graphic 93392 Version 2.0

Early repolarization end-QRS notch and slur terminology
(A) Illustration of the amplitudes J onset (Jo), J peak (Jp), and J termination (Jt), as well as durations D 1 and
D 2 , in relation to an end-QRS notch, as defined in the text.
(B) Illustration of Jp and Jt, as well as D 2 , in relation to an end-QRS slur.
Reproduced from: Macfarlane PW, Antzelevitch C, Haissaguerre M, et al. The Early Repolarization Pattern: A Consensus
Paper. J Am Coll Cardiol 2015; 66:470. Illustration used with the permission of Elsevier Inc. All rights reserved.

Early repolarization 12 lead ECG
Early repolarization manifest as inferior J-point slurring and lateral J-point notching, each >1
mm in two contiguous leads.

J or Osborn wave
ECG change with hypothermia, including presence of J wave.
Reproduced with permission from: Hickey R, De Caen A. Warming Procedures. In:

Textbook of Pediatric Emergency Procedures, 2nd ed, King C, Henretig FM (Eds),
Lippincott Williams & Wilkins, Philadelphia 2008. Copyright © 2008 Lippincott
Williams & Wilkins. www.lww.com.

Early repolarization 12-lead electrocardiogram
Marked inferior and lateral ER of 3 mm.
ER: early repolarization.

Action potentials in early repolarization
A disproportionately abbreviated epicardial action potential compared with the endocardial

action potential causes J-point elevation.

Action potential currents
Major cardiac ion currents and channels responsible for a ventricular action potential are shown with their common
name, abbreviation, and the gene and protein for the alpha subunit that forms the pore or transporter. The diagram
on the left shows the time course of amplitude of each current during the action potential, but does not accurately
reflect amplitudes relative to each of the other currents. This summary represents a ventricular myocyte, and lists
only the major ion channels. The currents and their molecular nature vary within regions of the ventricles, and in
atria, and other specialized cells such as nodal and Purkinje. Ion channels exist as part of multi-molecular
complexes including beta subunits and other associated regulatory proteins which are also not shown.
Courtesy of Jonathan C Makielski, MD, FACC.

Signal-averaged ECG in early repolarization
Signal-averaged ECG in a patient with ER demonstrating normal parameters (filtered QRS duration 124
milliseconds, duration of high frequency low amplitude signal less than 40 microvolts of 37 milliseconds, and
root mean square voltage in the terminal 40 milliseconds of 19 microvolts).

Approach for the acute management of ventricular arrhythmia
An algorithmic approach for the acute management of ventricular arrhythmia associated with genetic
disorders.
VF: ventricular fibrillation; PMVT: polymorphic ventricular tachycardia, possible torsade de pointes; ERS:
early repolarization syndrome.
* Refer to UpToDate text for details.

Contributor Disclosures
Andrew Krahn, MD Nothing to disclose Manoj Obeyesekere, MBBS Nothing to disclose Mark S Link,
MD Patent Holder: Tufts Medical Center [Chest wall protector]. Brian C Downey, MD, FACC Nothing to
disclose
Contributor disclosures are reviewed for conﬂicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform to
UpToDate standards of evidence.
Conﬂict of interest policy

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Official reprint from UpToDate®

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ECG findings — On the ECG, ER is defined as either:

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ER pattern versus ER syndrome — As noted above, ER is an ECG finding. Two terms,

PREVALENCE OF ER AND INCIDENCE OF IDIOPATHIC VF

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● In a population based case-cohort study of individuals of central-European descent (n = 6213,

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● Distribution and amplitude of ER

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● A high prevalence of ER in noncompaction cardiomyopathy patients has been reported,

● ER has been demonstrated to modify symptoms in patients with catecholaminergic polymorphic

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GENETIC BASIS AND INHERITANCE OF ER

Inheritance of ER pattern — The ER pattern may be sporadic or inherited, although first-degree

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MECHANISM OF ER AND IDIOPATHIC VENTRICULAR FIBRILLATION

ER mechanistically demonstrates some similarities to Brugada Syndrome and short QT syndrome

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There is controversy regarding whether the ER pattern represents repolarization or depolarization.

CLINICAL MANIFESTATIONS AND DIAGNOSIS

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ER syndrome causing VF may be diagnosed when:

● Other etiologies have been systematically excluded

ER syndrome causing VF is probable when:

● Other etiologies have been systematically excluded

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Treatment of ER syndrome with idiopathic VF — Among survivors of SCD due to idiopathic

Chronic treatment of ER syndrome — Chronic treatment of the ER syndrome should include an

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● In an in vitro pharmacological model, the phosphodiesterase III inhibitors cilostazol or milrinone

SOCIETY GUIDELINE LINKS

SUMMARY AND RECOMMENDATIONS

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● ER is an ECG finding. Two terms, distinguished by the presence or absence of symptomatic

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3. Macfarlane PW, Antzelevitch C, Haissaguerre M, et al. The Early Repolarization Pattern: A

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9. Surawicz B, Macfarlane PW. Inappropriate and confusing electrocardiographic terms: J-wave

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