Long QT Syndrome and Short QT Syndrome
作者： Wojciech Zareba , Iwona Cygankiewicz
DOI: 10.1016/J.PCAD.2008.10.006
关键词:
摘要: The long QT syndrome (LQTS) is a genetic disorder characterized by prolongation of the interval in electrocardiogram (ECG) and propensity to torsades de pointes ventricular tachycardia frequently leading syncope, cardiac arrest, or sudden death usually young otherwise healthy individuals. caused mutations predominantly potassium sodium ion channel genes channel-related proteins positive overcharge myocardial cell with consequent heterogeneous repolarization various layers regions myocardium. These conditions facilitate early after-depolarization reentry phenomena underlying development polymorphic observed patients LQTS. Obtaining detailed patient history regarding events his/her family members combined careful interpretation standard 12-lead ECG (with precise measurement all available ECGs evaluation T-wave morphology) sufficient diagnose Genetic testing plays an important role particularly useful cases nondiagnostic borderline findings. clinical course LQTS depends on extent QTc modulated age, sex, genotype. beta-Blockers remain therapy choice for LQTS, but implantation cardioverter defibrillator increasingly used high-risk patients.
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