Short QT syndrome
ICD-10 I49.9 · ICD-11 BC65.2

Treatment of Short QT Syndrome After Aborted Cardiac Arrest, Sustained Ventricular Tachycardia, or Arrhythmic Syncope

This protocol applies to patients with confirmed Short QT syndrome (SQTS) who have experienced at least one of the following high-risk arrhythmic events:

  • Survivor of an aborted cardiac arrest
  • Documented spontaneous sustained ventricular tachycardia
  • Arrhythmic syncope

Each of these presentations places the patient in a high-risk category requiring structured, guideline-directed management.


Management in this setting includes antiarrhythmic pharmacotherapy — quinidine is part of the approach, with monitoring required for excessive QT prolongation. The full protocol covers the complete algorithm, sequencing, and all relevant clinical considerations.
References
DOI: 10.1093/eurheartj/ehac262
ICD implantation is recommended in patients with a diagnosis of SQTS who: (a) are survivors of an aborted CA and/or (b) have documented spontaneous sustained VT.
ICD implantation should be considered in SQTS patients with arrhythmic syncope.
Quinidine may be considered in (a) SQTS patients who qualify for an ICD but present a contraindication to the ICD or refuse it, and (b) asymptomatic SQTS patients and a family history of SCD.
Quinidine is currently the best supported AAD, but should be monitored for excessive QT prolongation, while isoprenaline may be considered in electrical storm.
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