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Short QT Syndrome — EECC MCQ

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HardArrhythmia & ElectrophysiologyShort QT SyndromeEECC

A 40-year-old woman is referred after her sister was diagnosed with Short QT Syndrome (SQTS). Her QTc is 310 ms on ECG. She is asymptomatic and has no family history of sudden death beyond her sister's diagnosis. What is the arrhythmic risk and management?

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Correct answer: E — SQTS is associated with increased risk of AF and VF; ICD is recommended for symptomatic patients; quinidine may lengthen QT and is used in asymptomatic patients who decline ICD

Short QT syndrome (SQTS) is a rare inherited channelopathy characterised by abnormally short QT interval (QTc ≤340 ms, with diagnosis strongly suggested at ≤320 ms) and increased susceptibility to AF and VF due to shortened refractory periods. The 2022 ESC VA/SCD Guidelines recommend: ICD for survivors of cardiac arrest or with documented sustained VT/VF (Class I); ICD may be considered in asymptomatic patients with very short QTc and a family history of SCD (Class IIb). Quinidine (a class IA antiarrhythmic) uniquely prolongs the QT interval in SQTS (particularly in KCNH2 mutations) and may be used as pharmacological therapy when ICD is declined or as an adjunct. Other antiarrhythmics do not effectively lengthen the QT in SQTS.

Reference: ESC (2022): Guidelines on VA and SCD Prevention