Risk stratification in long QT syndrome: Are we finally getting closer to a mutation-specific assessment of an individual patient's arrhythmogenic risk?

摘要

Congenital long QT syndrome (LQTS) is a heterogeneous ion channelopathy in which a prolonged cardiac repolariza-tion predisposes patients to polymorphic ventricular tachycardia, syncope, and sudden cardiac death (SCD). The individual arrhythmogenic risk, however, varies pronouncedly between different patients with LQTS, even in those carrying the same mutation. The assessment of the risk of an individual patient with LQTS thus remains a difficult task in daily practice: even though risk stratification protocols are useful, one needs to carefully balance aggressiveness of treatment (eg, medication with beta-blockers, implantable cardioverter-defibrillator implantation, and/or sympathec-tomy) with the presumed risk of the individual patient with LQTS. In addition, the need for lifelong therapy in these young patients needs to be considered.