Short QT syndrome

Short QT syndrome is a very rare genetic disease of the electrical system of the heart. It takes its name from a characteristic finding on the electrocardiogram, a shortened QT interval, and it carries an increased risk of abnormal heart rhythms and sudden cardiac death. The condition is caused by mutations in genes that encode ion channels, which shorten the cardiac action potential, and it appears to be inherited in an autosomal dominant pattern. Diagnosis is made with a 12-lead electrocardiogram. Treatment options include an implantable cardioverter-defibrillator or medication such as quinidine. Short QT syndrome was first described in 2000, and the first genetic mutation associated with it was identified in 2004, so it is one of the more recently recognised inherited arrhythmia disorders.