Short QT Syndrome

The short QT syndrome (SQTS) is a rare cardiac ion channel disease predisposing to ventricular arrhythmias (VAs) and sudden cardiac death (SCD) in the young [1]. The signature feature of SQTS is the presence of a constantly short QT interval at ECG in the absence of structural cardiac abnormalities. First described in 2000 [2], SQTS was recognised as a potentially inheritable condition predisposing to SCD in 2003 [3], and the demonstration of the first genetic mutation soon followed [4]. In the last 20 years, approximately 200 affected subjects have been reported in the lit- erature worldwide, with few case series published so far [5–8]. Data from the Euro Short Registry published in 2006 [5] and 2011 [6] helped in outlining the diagnostic criteria, clinical manifestations and therapeutic strategies for SQTS, which are sum- marised in the current consensus documents and guidelines on the management of inherited primary arrhythmia syndromes and the prevention of SCD [1, 9].