Early repolarization syndrome: A cause of sudden cardiac death

Table 1 Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society consensus statement on the diagnosis and management of primary inherited arrhythmia syndromes recommended criteria for the diagnosis of early repolarization

ER expert consensus recommendations on early repolarization diagnosis
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
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
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

ER: Early repolarization; ECG: Electrocardiogram; SCD: Sudden cardiac death. Reproduced from ref.[50], with permission from the publisher.

Table 2 Conditions with J-wave on the electrocardiogram

Conditions with predominant J-waves
Hypothermia
Hypercalcaemia
Hyperkalaemia
Vasospastic angina
Brugada syndrome
Early repolarization syndrome
Short QT syndrome
Hypoxia
Acidosis
Pulmonary embolism
Arrhythmogenic right ventricular cardiomyopathy
Subarachnoid haemorrhage

Reproduced from ref.[50], with permission from the publisher.

Table 3 Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society consensus statement on the diagnosis and management of primary inherited arrhythmia syndromes recommendations for therapeutic interventions in early repolarization syndrome

Expert consensus recommendations on early repolarization therapeutic interventions
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 or more 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

ER: Early repolarization; ECG: Electrocardiogram; ICD: Implantable cardioverter-defibrillator.