CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR TACHYCARDIA: THE CLINICIAN'S PERSPECTIVE


Martin J. LaPage, M.D., Michigan, USA

Catecholaminergic polymorphic polymorphic ventricular tachycardia (CPVT) is a potentially lethal, genetic channelopathy syndrome characterized by ventricular arrhythmias occurring primarily during exercise. Patients typically present with syncope or seizures and initial misdiagnosis is common because baseline cardiac testing is normal. Untreated CPVT imposes a 30-50% mortality risk by age 30. The past decade has seen important advances in the genetic diagnosis and treatment of this disease which can significantly decrease the risk of sudden death. This presentation will focus on the recognition and accurate diagnosis of CPVT in addition to the pathophysiology and appropriate treatment.