cardiovascular disorders
Catecholaminergic Polymorphic Ventricular Tachycardia
Treatment of CPVT
Exercise restriction Beta blockade Sometimes an implantable cardioverter-defibrillator (ICD) Rarely left cardiac sympathetic denervation All patients are advised to avoid strenuous exercise (eg, competitive sports). Particularly those who will not accept exercise restrictions should be counseled on the need for appropriate cautions (eg, availability of an automated external defibrillator during training and competition). For patients who have had symptoms (eg, syncope, cardiac arrest) or who have demonstrated VF or sustained VT, the primary treatment is high doses of a long-acting beta-blocker, preferably one without intrinsic sympathomimetic activity (eg,nadolol, slow-releasepropranolol). If beta blockade is ineffective,flecainidemay be useful both for its sodium channel and ryanodine receptor blocking effects. Implantable cardioverter-defibrillators (ICDs) are used for patients with CPVT who survived cardiac arrest or who have VT despite antiarrhythmic drug therapy. Refractory cases are treated with left cardiac sympathetic denervation and/or arrhythmia substrate ablation. Follow-up typically involves repeated exercise testing, but the predictive value is only moderate. Asymptomatic patients (including relatives identified by genetic testing) should also moderate their physical activity and receive beta-blocker therapy. 