Recurring Tachycardia and Syncope in a Young Person

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac arrhythmia in the absence of structural cardiopathy with a strong potential for sudden cardiac death (SCD). A patient with a worrisome family history of SCD and genetically proven CPVT in various family members was found to have CPVT with exaggerated cathecolaminergic burden and stressinduced complex cardiac arrhythmias. The arrhythmias are mediated by delayed after-depolarisation. The antiarrhythmic effect of the β-blocker metoprolol was insufficient. The patient refused other antiarrhythmic drugs. He received an implantable cardioverter defibrillator (ICD), which was effective in treating the recurring arrhythmia. Vigorous physical activity and high emotional stress must be avoided by these patients. The case underlines the importance of ICD in patients with recurring syncopes, a strong family history of SCD with a genetically proven CPVT in various family members.