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[Not so Benign] Early Repolarisation in
10 mins – a ‘quick-lit-review’.
Simon Mark Daley (2018)
Early Repolarisation (ER)…
▪ Also historically referred to as ‘high take off’ - this term is outdated.
▪ Up to 15% of chest pain presentations to the ED will have ER.
▪ More common <50yrs, less common >70yrs.
▪ Physiological basis remains poorly understood.
▪ Historically thought to be benign. In the past 20 years multiple
studies have shown a link to threatening arrhythmia and sudden
cardiac death (SCD).
▪ Risk stratification remains challenging & controversial, although
increasingly less so.
Is there an ER definition?
▪ A 2015 consensus paper recommended that a new definition was
urgently needed. It proposed the below criteria, which subsequently
was reiterated by a further consensus meeting in 2016;
▪ ERP is present if the following criteria are met;
1. There is end-QRS notch or slur on the downslope of a prominent R-
wave. If there is a notch, it should lie entirely above the baseline. The
onset of a slur must also be above the baseline.
2. J-point is >0.1mV in 2 or more contiguous leads, excluding v1-v3.
3. QRS duration is <120ms.
ST elevation in the absence of a notched or slurred J-point should
not be described as ER.
Macfarlane et al (2015) and Antzelevitch et al (2016)
?ERP in v1-v3
▪ When ERP/non-specific ST-
segment elevation is present in
these leads, give careful
consideration to differential
diagnoses.
Antzelevitch et al (2016)
Terminology / classification…
▪ The accuracy of categorisation is of importance,
and should be standardised.
▪ If the ST-segment is upward sloping and
followed by an upright T-wave, the pattern should
be described as “early repolarization with an
ascending ST segment.”
▪ If the ST-segment is horizontal or downward
sloping, the pattern should be described as “early
repolarization with a horizontal or descending ST
segment.”
▪ The leads in which the notching or slurring occurs
should be used as part of the description, so that,
for example, a complete report might state, “early
repolarization with descending ST-segment in
leads II, III, and aVF.” Macfarlane et al (2015)
ER pattern / ER syndrome…
▪ ER syndrome is diagnosed when
there is ERP in the inferior/lateral
leads presenting with aborted cardiac
arrest, VF or polymorphic VT.
Antzelevitch et al (2016)Macfarlane et al (2015)
Pathophysiology of ERS (&BrS)…
▪ Early repolarisation syndrome lies on a spectrum with BrS; Many
clinical similarities – suggesting similar pathophysiology.
▪ Males predominate both syndromes (71% of BrS & 80% of ERS).
▪ Incidence of VF highest in third decade of life; ?linked to testosterone.
▪ ERP is prevalent in Africans/African-Americans, but apparently not
associated with high risk.
Risk stratification…
▪ Studies have shown that ER – especially in the inferior leads – predicts cardiac
and arrhythmic death.
▪ The incidental discovery of a J-wave should not be interpreted as a marker of ‘high
risk’ for SCD since the odds are extremely low. Viskin et al (2014).
▪ Presence of a J-wave on the ECG may increase the probability of VF from
3.4:100,000 to 11:100,000. Rosso et al (2011).
Antzelevitchetal(2016)
Management;
▪ ßeta-blockers can suppress electrical storms and associated J-wave manifestations.
▪ Long-term therapy using quinidine, bepridil, denopamine, & cilostazol is reported to
suppress the development of VF/VT in both.
Antzelevitchetal(2016)
Antzelevitch et al (2016)
Take away points;
▪ ER is no longer considered benign.
▪ ERS lies on a spectrum with BrS.
▪ ERP in the anterior leads appears more likely to be ‘benign’, with
inferior/lateral ERP more likely to be high risk. Care must still be
taken when labelling anterior ER/non-specific STE as BER.
▪ Symptomatic ERS is an indication for ICD implantation.
▪ In the absence of syncope, or a strong family history of sudden
cardiac death (SCD), the finding of the ERP does not merit further
investigation. Asymptomatic ERS should receive ‘close’ FU.

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Early repolarisation in 10 mins - A quick lit review

  • 1. [Not so Benign] Early Repolarisation in 10 mins – a ‘quick-lit-review’. Simon Mark Daley (2018)
  • 2. Early Repolarisation (ER)… ▪ Also historically referred to as ‘high take off’ - this term is outdated. ▪ Up to 15% of chest pain presentations to the ED will have ER. ▪ More common <50yrs, less common >70yrs. ▪ Physiological basis remains poorly understood. ▪ Historically thought to be benign. In the past 20 years multiple studies have shown a link to threatening arrhythmia and sudden cardiac death (SCD). ▪ Risk stratification remains challenging & controversial, although increasingly less so.
  • 3. Is there an ER definition? ▪ A 2015 consensus paper recommended that a new definition was urgently needed. It proposed the below criteria, which subsequently was reiterated by a further consensus meeting in 2016; ▪ ERP is present if the following criteria are met; 1. There is end-QRS notch or slur on the downslope of a prominent R- wave. If there is a notch, it should lie entirely above the baseline. The onset of a slur must also be above the baseline. 2. J-point is >0.1mV in 2 or more contiguous leads, excluding v1-v3. 3. QRS duration is <120ms. ST elevation in the absence of a notched or slurred J-point should not be described as ER. Macfarlane et al (2015) and Antzelevitch et al (2016)
  • 4. ?ERP in v1-v3 ▪ When ERP/non-specific ST- segment elevation is present in these leads, give careful consideration to differential diagnoses. Antzelevitch et al (2016)
  • 5. Terminology / classification… ▪ The accuracy of categorisation is of importance, and should be standardised. ▪ If the ST-segment is upward sloping and followed by an upright T-wave, the pattern should be described as “early repolarization with an ascending ST segment.” ▪ If the ST-segment is horizontal or downward sloping, the pattern should be described as “early repolarization with a horizontal or descending ST segment.” ▪ The leads in which the notching or slurring occurs should be used as part of the description, so that, for example, a complete report might state, “early repolarization with descending ST-segment in leads II, III, and aVF.” Macfarlane et al (2015)
  • 6. ER pattern / ER syndrome… ▪ ER syndrome is diagnosed when there is ERP in the inferior/lateral leads presenting with aborted cardiac arrest, VF or polymorphic VT. Antzelevitch et al (2016)Macfarlane et al (2015)
  • 7. Pathophysiology of ERS (&BrS)… ▪ Early repolarisation syndrome lies on a spectrum with BrS; Many clinical similarities – suggesting similar pathophysiology. ▪ Males predominate both syndromes (71% of BrS & 80% of ERS). ▪ Incidence of VF highest in third decade of life; ?linked to testosterone. ▪ ERP is prevalent in Africans/African-Americans, but apparently not associated with high risk.
  • 8. Risk stratification… ▪ Studies have shown that ER – especially in the inferior leads – predicts cardiac and arrhythmic death. ▪ The incidental discovery of a J-wave should not be interpreted as a marker of ‘high risk’ for SCD since the odds are extremely low. Viskin et al (2014). ▪ Presence of a J-wave on the ECG may increase the probability of VF from 3.4:100,000 to 11:100,000. Rosso et al (2011). Antzelevitchetal(2016)
  • 9. Management; ▪ ßeta-blockers can suppress electrical storms and associated J-wave manifestations. ▪ Long-term therapy using quinidine, bepridil, denopamine, & cilostazol is reported to suppress the development of VF/VT in both. Antzelevitchetal(2016) Antzelevitch et al (2016)
  • 10. Take away points; ▪ ER is no longer considered benign. ▪ ERS lies on a spectrum with BrS. ▪ ERP in the anterior leads appears more likely to be ‘benign’, with inferior/lateral ERP more likely to be high risk. Care must still be taken when labelling anterior ER/non-specific STE as BER. ▪ Symptomatic ERS is an indication for ICD implantation. ▪ In the absence of syncope, or a strong family history of sudden cardiac death (SCD), the finding of the ERP does not merit further investigation. Asymptomatic ERS should receive ‘close’ FU.