Address for Correspondence:
Dr. Frederic Sacher, MD, Clinical Electrophysiologist, Cardiac
Arrhythmias Department, LIRYC Institute, Hopital Cardiologique
Haut Leveque, Avenue de Magellan - 33604 Pessac -
France. E-mail: frederic.sacher@chu-bordeaux.fr
Keywords: Early Repolarization Syndrome, sudden
cardiac death
The electrocardiographic pattern of early repolarization (ER)
is common, with a particularly high prevalence reported
amongst athletes and adolescents. It has long been associated
with benign outcome [1-3]. Recently, an association between
inferolateral ER pattern and sudden cardiac death (SCD) has
been established by different groups [4-7]. Population-based
studies have also reported an increased mortality rate among
patients with inferolateral ER pattern compared to controls
[7-9]. To bring back together these differences, it is
important to focus on the definition of ER pattern used in
these different studies as well as the population included.
The definition of ER pattern associated with sudden cardiac
death was the presence of J point elevation more than or equal
to 0.1mV in at least 2 contiguous inferior and/or lateral
leads of a standard 12-lead ECG and not ST elevation as it was
often the case in the studies with benign outcome. Any study
dealing with ER should clearly indicate the definition used.
Otherwise it cannot be interpreted. Talking about definition,
ER syndrome is an ER pattern (as defined above) associated
with symptoms (syncope or aborted SCD) and/or familial history
of SCD as mentioned in the last HRS/EHRA/APHRS Expert
Consensus Statement on the Diagnosis and Management of
Patients with Inherited Primary Arrhythmia Syndromes [10]. It
is important to recognise that having only an ER pattern is
not a disease.
Another potential difference between the studies focusing on
ER is the studied populations. Studies with a correlation
between ER pattern and SCD included mainly Caucasian or Asian
and very little African, African/American nor athletes.
Therefore, their findings cannot be extrapolated to these
populations.
Based on the different studies linking ER pattern and SCD,
only a small minority of patients with this pattern will
experience SCD, while the majority will remain asymptomatic.
Therefore, the identification of this minority of patients
represents a significant challenge. Currently, criteria for
malignant ER pattern rely on 12 leads ECG parameters on top of
arrhythmic syncope. The amplitude of J point more than 0.2mV
and a descending ST segment are the 2 main pejorative
parameters [5,7,11-13]. The presence of the ER pattern in
inferior vs lateral leads, notch vs slur and QRS fragmentation
have been reported as pejorative criteria but are still
controversial.
Concerning risk stratification, it seems now clear that
programmed ventricular stimulation has no interest in
asymptomatic patient and should not be done [14]. Currently,
asymptomatic persons with ER pattern should not be treated.
However close follow-up is warranted in case of J point
elevation more than 0.2mV with horizontal/descending ST
segment. In patients with arrhythmic syncope, an implantable
cardioverter defibrillator (ICD) may be considered (based on
the expert consensus statement). In clinical practice, syncope
may be of undetermined origin; ICD vs loop recorder
implantation is then a case by case decision. Of note,
patients with resuscitated SCD and ER pattern have a
recurrence risk x2 compared to the same population without ER
pattern. In case of recurrent ICD shocks, isoproterenol may
halt arrhythmic storm and quinidine may prevent from recurrent
ventricular fibrillation episodes [15,16].
Because 5% of a caucasian population may present this pattern
with only 1/2500 of them at risk of ventricular fibrillation,
better tools for risk stratification are necessary. However it
is crucial to recognise this pattern in patients with a
familial history of SCD, patients with syncope or patients
with dramatic J point elevation and descending/horizontal ST
segment.
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