Version June 2024
| 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. |
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