Channelopathies: Brugada syndrome, long QT syndrome, short QT syndrome, and CPVT.

摘要: In approximately 10-20% of all sudden deaths, no structural cardiac abnormalities can be identified. Important potential causes deaths in the absence heart disease are primary electrical diseases such as Brugada syndrome, long QT syndrome (LQTS), short (SQTS), and catecholaminergic polymorphic ventricular tachyarrhythmias (CPVT). The resting ECG under exercise pivotal for diagnosis ion channel diseases. Molecular genetic screening reveal underlying mutations a variable degree among up to 70% (LQTS) may identify individuals with incomplete penetration disease. patients diseases, specific clinical triggers arrhythmic events syncope or death have been identified including exercise, strenuous activity, auditory stimuli, increased vagal tone. Young, otherwise healthy likely involved sports activity. Therefore, special attention has given advise these patients. Competitive vigorous contraindications almost Even recreational avoided phenotypically overt silent gene carriers depending on