The short QT syndrome is associated with a family history of sudden death.


Gene affected: HERG (KCNH2) which affects the potassium channel. Several mutations have been identified.


Clinical features:

(1) family history of sudden death which may occur at any age, including during infancy

(2) absence of structural heart disease

(3) syncope


ECG and electrophysiologic features:

(1) short QT interval (QTc < 360 ms according to Schimpf et al; <= 300 ms according to Gaita et al)

(2) atrial fibrillation and/or ventricular tachyarrhythmias

(3) very short ventricular refractory periods on electrophysiologic studies

(4) increased dispersion of repolarization

(5) ability to induce malignant ventricular arrhythmias during electrophysiologic studies



(1) ICD placement in an adult

(2) drug therapy with quinidine or disopyramide


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