ECG quiz – Discussion

Findings and diagnosis?

Early repolarization syndrome

Early repolarization syndromeConcave upwards ST segment elevation with a notch at the end of the QRS complex (J point) or a slurring of the terminal QRS seen in lateral leads is characteristic of early repolarization syndrome (ERPS). Till recently ERPS was considered as a benign condition, only thought of in the differential diagnosis of ST elevation myocardial infarction. But a paper by Haissaguerre  M et al in NEJM [1] in 2008 brought ERPS into limelight as a potential arrhythmogenic condition. Actually the same group had highlighted on the new syndrome the previous year in the Journal of Interventional Cardiac Electrophysiology [2].

In an article by Frederic Sacher and Michel Haissaguerre in BMH Medical Journal [3], they highlighted on the need for specific definition for ERPS. They also mentioned that having an ERPS pattern is not a disease by itself. But it is crucial to give importance when there is a family history of sudden cardiac death and in patients with syncope having dramatic J point elevation and a descending/horizontal ST segment. They mentioned that even though 5% of Caucasian population may have this ECG pattern, only 1/2500 are at risk of ventricular fibrillation.

Another article by the same group of authors has given a few ECG examples in which ventricular fibrillation occurred after an ectopic beat in those with early repolarization syndrome [4].

Reference

  1. Haissaguerre M, Derval N, Sacher F, Jesel L, Deisenhofer I, de Roy L, Pasquie JL, Nogami A, Babuty D, Yli-Mayry S, De Chillou C, Scanu P, Mabo P, Matsuo S, Probst V, Le Scouarnec S, Defaye P, Schlaepfer J, Rostock T, Lacroix D, Lamaison D, Lavergne T, Aizawa Y, Englund A, Anselme F, O’Neill M, Hocini M, Lim KT, Knecht S, Veenhuyzen GD, Bordachar P, Chauvin M, Jais P, Coureau G, Chene G, Klein GJ, Clementy J. Sudden cardiac arrest associated with early repolarization. N Engl J Med. 2008; 358: 2016-2023.
  2. Haissaguerre M, Sacher F, Derval N, Jesel L, Deisenhofer I, de Roy L, Pasquie JL, Nogami A, Babuty D, Yli-Mayry S, Anselme F, Aizawa Y, Scanu P, Mabo P, Matsuo S, Probst V, Lavergne T, Defaye P, Maury P, Lamaison D, Hocini M, Lim KT, Veenhuyzen G, Bordachar P, Jais P, Klein G, Clementy J. Early Repolarization in the Inferolateral Leads: A New Syndrome Associated with Sudden Cardiac Death. J Interv Card Electrophysiol. 2007; 18: 281.
  3. Sacher F, Haissaguerre M. Early Repolarization Syndrome. BMH Medical Journal 2015;2(2):28-30.
  4. Bourier F, Denis A, Cheniti G, Lam A, Vlachos K, Takigawa M, Kitamura T, Frontera A, Duchateau J, Pambrun T, Klotz N, Derval N, Sacher F, Jais P, Haissaguerre M, Hocini M. Early Repolarization Syndrome: Diagnostic and Therapeutic Approach. Front Cardiovasc Med. 2018 Nov 27;5:169. doi: 10.3389/fcvm.2018.00169. PMID: 30542653; PMCID: PMC6278243.

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