Electrophysiological characteristic of a patient exhibiting the short-coupled variant of torsade de pointes

Idiopathic Ventricular Fibrillation “Le Syndrome d'Haïssaguerre” and the Fear of J Waves

“…In an instructive case (14), verapamil abolished the shortcoupled extrasystoles that triggered VF (without prolonging the very short ventricular refractory period), while nifekalant prolonged the refractory period without suppressing the triggers of arrhythmia (14). Nonetheless, failure of verapamil has also been the rule in our experience (3).…”

Short-coupled variant of torsade de pointes

“…3 The short-coupled variant of TdP is a rarely described cause of ventricular tachycardia. [4][5][6] Literature describes healthy young patients who often have a positive family history for sudden cardiac death. [5][6][7] Yet, the responsible gene(s) have not yet been resolved.…”

“…[4][5][6] Literature describes healthy young patients who often have a positive family history for sudden cardiac death. [5][6][7] Yet, the responsible gene(s) have not yet been resolved. In one patient, mutations in the cardiac sodium channel encoding gene SCN5A (analysed because of the differential diagnosis of Brugada syndrome) were not found.…”

“…In one patient, mutations in the cardiac sodium channel encoding gene SCN5A (analysed because of the differential diagnosis of Brugada syndrome) were not found. 6 The most common first symptom is syncope. The ECG displays typical TdP with a remarkably short coupling interval (always less then 300 ms) of the first TdP beat 5 .…”

“…Yet, one study suggested that the I Kr blocker nifekalant may suppress arrhythmia inducibility by prolonging repolarisation, arguing against a causal role of EADs. 6 Either way, verapamil does not seem to reduce the risk of sudden cardiac death; therefore ICD implantation is advised. 5 Leenhart et al described 14 patients with the shortcoupled variant of TdP.…”