A Case Report Of Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia syndrome or so-called channelopathy caused by mutations in genes coding for sarcoplasmic calcium channel. CPVT is characterized by the occurrence of bidirectional or polymorphic ventricular tachycardia induced during exercise or emotional stress. CPVT usually occurs in children and adolescents with an estimated prevalence of1:10000. The mortality of CPVT is extremely high, with an estimated8-year cadiac event rates over30%. The gold standar for the diagnosis of CPVT is bidirectional or polymorphic ventricular arrhythmia induced in exercise-stress test. Beta-blockers are the mainstay for treating CPVT. Other therapeutic options include calcium-channel blockers, flecainide and left cardiac sympathetic denervation surgery. Implantable cardioverter defibrillator (ICD) therapy is significantly important for preventing sudden cardiac death in CPVT patients. Here we report a young female with family history of sudden cardiac death and with recurrent episodes of palpitation and syncope after exercise or emotional stress. CPVT was finally diagnosed after careful history-taking and positive exercise-stress test. The patient received oral β-blockers and her symptoms are well controlled. In our text, we also reviewed current understanding of pathogenesis, genetic mutation, pathophysiology, clinical manifestation andherapeutic options of CPVT.