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Surgical Approach in Congenital Long QT Interval Syndrome Patients

Summary Long QT syndrome is a genetically determined clinical condition that can lead to sudden cardiac death, life–threatening arrhythmias, typically ventricular tachycardia – Torsades de Pointes in young, otherwise healthy, adults and children. Congenital long QT syndrome is the most common cause of sudden death in young adults with structurally normal heart. There are several studies, which introduce us to gene mutation types, responsible for this disease. At this point 17 types of LQTS gene mutations are recognized, most patients present with the first 3 LQTS gene mutations: KCNQ1, KCNH2, and SCN5A. Secondary factors like electrolyte disbalance, dietary restrictions, and specific drugs may also cause QT interval prolongation. It is important to rule out avoidable causes, before further evaluation of congenital disease. Several treatment options are used in daily practice, which also includes a surgical approach. Although not so often used and seen, surgical technique has positive results – recognized by both doctors and patients.