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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic disorder characterized by symptomatic ventricular tachycardia (VT) associated with adrenergic activation in patients with no structural heart disease.
More than 60% of patients have an episode of syncope or cardiac arrest before the age of 20 years. Life-threatening arrhythmias are recurrent. In addition to malignant VT, patients may have other types of arrhythmia, such as atrial fibrillation.
The key symptoms include syncope caused by fast polymorphic VT, cardiac arrest, or sudden cardiac death, most commonly triggered by exercise or emotions.
Diagnosis is based on identifying the arrhythmia typical for CPVT: a fast, polymorphic, frequently bidirectional VT (beat-to-beat changes in the QRS polarity from negative to positive or vice versa), which is most easily triggered by an exercise test.
Classification of antiarrhythmic drugs: see Table 3.4-1.
Antiarrhythmic agents: see Table 3.4-2.
Avoidance of exercise, treatment with beta-blockers. Placement of an implantable cardioverter-defibrillator (ICD) is indicated in patients with a history of cardiac arrest as well as in those with syncope or VT despite beta-blocker treatment.