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Short QT Syndrome (SQTS)
Short QT syndrome (SQTS) is a heart disorder that affects the cardiac rhythm due to abnormal functioning of ion channel proteins. It is characterized by shortening of the QT interval on ECG and paroxysmal atrial and ventricular tachyarrhythmias (episodes of rapid heartbeats originating from the heart chambers). SQTS is associated with an increased risk of atrial fibrillation and sudden cardiac death, resulting from an accelerated cardiac atrial and ventricular repolarization. Fewer than 30 cases of short QT syndrome have been published since the condition was first described in 2000.
The diagnosis of SQTS is based on clinical history, ECG findings and family history. The disorder may manifest in any stage of life, sometimes as early as infancy. Patients may have a history of atrial fibrillation, or less likely, syncope. SQTS may be present even in the absence of any clinical symptoms, and in some patients sudden cardiac death occurs without any preceding symptoms and without an identifiable cause at autopsy. Autosomal dominant SQTS may also underlie some cases of sudden infant death syndrome (SIDS).