Short QT syndrome: Review [Kisa QT sendromu]

Abstract

Short QT syndrome (SQTS) is a gene-related arrhythmogenic syndrome harbouring a large spectrum of symptoms ranging from mild palpitations to sudden cardiac death. The disease is known for an abnormally short QT interval (<300 ms) and a propensity to atrial fibrillation, sudden cardiac death or both. As in the case of long QT syndrome, more than one relevant genetic mutation has been identified that can lead to electrocardiographic changes. The mutation of genes (KCNH2, KCNQ1, and KCNJ2) encoding for cardiac potassium channels plays a central role in SQTS. Electrocardiography is the primary important step in the diagnosis (short QT interval along with T wave changes), but ECG findings may be easily ignored. Several cases, especially among children, have probably been overlooked, since the shortness of the QT interval becomes apparent only at heart rates less than 80 beats/min. Treatment of the syndrome is still controversial. Some specific antiarrhythmic drugs and an implantable converter/defibrillator (ICD) have been considered as main therapeutic strategies. ICD implantation may be a life-saving procedure due to the presence of sudden cardiac death risk in patients with SQTS, but ICD-related problems such as inappropriate shock deliveries due to oversensing of prominent T waves have made medical therapy an alternative option. Copyright © 2011 by Türkiye Klinikleri.