Diagnosis
Specific diagnostic criteria for early repolarisation pattern and ERS were presented by the Heart Rhythm Society (HRS), European Heart Rhythm Association (EHRA) and Asia Pacific Heart Rhythm Society (APHRS) in 2013, as follows.24
ERS is diagnosed in:
1. 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. A 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; and
3. The presence of J-point elevation ≥1 mm in ≥2 contiguous inferior and/or lateral leads of a standard 12-lead ECG.
In addition, specific repolarisation patterns that have been previously discussed should be also taken into account. The Brugada syndrome is characterised by J-point or ST-segment elevation in the right precordial leads, and approximately 12 % of patients display typical early repolarisation abnormalities. However, it is typical that the ST-segment elevation is augmented in the right precordial leads by sodium-channel blockers; whereas in ERS, the early repolarisation pattern is usually attenuated.25
Therapy
The risk stratification and optimum management of these patients are not well defined and the recognition of the truly malignant forms is difficult. Electrophysiology testing does not appear useful for risk stratification. VF is infrequently induced (22 %) and has no predictive value for ICD therapy.26 Patients with aborted sudden death in the absence of identifiable cause (idiopathic VF) are treated with ICD. Ablation of idiopathic VF, targeted to short coupled ventricular premature beats that originate predominantly from the Purkinje system and the right ventricular outflow track and trigger VF, has also been reported.27 According to the 2013 HRS/EHRA/APHRS statement, ICD is indicated only in patients who have survived a cardiac arrest (I) and it might be considered (IIb) 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. Quinidine may also be used in addition to ICD (IIa), as well as isoproterenol to suppress electrical storms (IIa).24