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Early Repolarisation Syndrome — EECC MCQ

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HardArrhythmia & ElectrophysiologyEarly Repolarisation SyndromeEECC

A 55-year-old man with a structurally normal heart has recurrent episodes of polymorphic VT occurring during sleep. His baseline ECG shows early repolarisation (J-point elevation) in the inferior leads. The episodes are reproducibly triggered by a pause followed by a short-long-short sequence. What is the likely diagnosis?

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Correct answer: DEarly repolarisation syndrome (ERS) with malignant arrhythmias — J-point elevation in inferior/lateral leads combined with VF/polymorphic VT constitutes a high-risk phenotype; isoproterenol acutely and quinidine chronically can suppress arrhythmias

Early repolarisation (ER) pattern (J-point elevation ≥1 mm in inferior and/or lateral leads) is present in 5-10% of the population and is usually benign. However, a small subset develops malignant arrhythmias (VF, polymorphic VT), termed early repolarisation syndrome (ERS). High-risk features include: J-point elevation ≥2 mm, horizontal/descending ST segment (not upsloping), inferior lead involvement, and documentation of VF. The 2022 ESC VA/SCD Guidelines recommend: (1) ICD for ERS survivors of cardiac arrest (Class I); (2) isoproterenol for acute VF storm (augments INa, suppresses pause-dependent VF); (3) quinidine for chronic suppression (Class IIa — blocks Ito current responsible for the J-wave); (4) avoid class IC AADs and vagotonic states. CPVT is distinguished by exercise-triggered (not pause-dependent) arrhythmias.

Reference: ESC (2022): VA/SCD Guidelines