1. CPVT: Arrhythmogenesis, Therapeutic Management, and Future Perspectives. A Brief Review of the Literature
- Author
-
Theofilos M. Kolettis, J Sieira, Giulio Conte, Giuseppe Ciconte, Carlo de Asmundis, Pedro Brugada, Giacomo di Giovanni, Gian Battista Chierchia, Dimitrios N. Lysitsas, Giannis G. Baltogiannis, Cardiology, Faculty of Medicine and Pharmacy, Heartrhythmmanagement, Clinical sciences, Cardio-vascular diseases, University of Zurich, and Baltogiannis, Giannis G
- Subjects
0301 basic medicine ,medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,CPVT ,sudden death ,610 Medicine & health ,Review ,risk stratification ,030204 cardiovascular system & hematology ,Cardiovascular Medicine ,Catecholaminergic polymorphic ventricular tachycardia ,Ventricular tachycardia ,Sudden death ,Ryanodine receptor 2 ,11171 Cardiocentro Ticino ,2705 Cardiology and Cardiovascular Medicine ,Sudden cardiac death ,03 medical and health sciences ,0302 clinical medicine ,Sodium channel blocker ,Internal medicine ,medicine ,genes ,Flecainide ,business.industry ,Ryanodine receptor ,medicine.disease ,channelopathies ,030104 developmental biology ,lcsh:RC666-701 ,Cardiology ,cardiovascular system ,Cardiology and Cardiovascular Medicine ,business ,arrhythmias ,medicine.drug - Abstract
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a primary electrical disease characterized by a normal resting electrocardiogram and induction of malignant arrhythmias during adrenergic stress leading to syncope or sudden cardiac death (SCD). CPVT is caused by mutations in the cardiac ryanodine receptor (RyR2) or in the sarcoplasmic reticulum protein calsequestrin 2 genes (CASQ2). The RyR2 mutations are responsible for the autosomal dominant form of CPVT, while CASQ2 mutations are rare and account for the recessive form. These mutations cause a substantial inballance in the homeostasis of intracellular calcium resulting in polymorphic ventricular tachycardia through triggered activity. Beta blockers were for years the cornerstone of therapy in these patients. Sodium channel blockers, especially flecainide, have an additive role in those not responding in beta blockade. Implantation of defibrillators needs a meticulous evaluation since inappropriate shocks may lead to electrical storm. Finally, cardiac sympathetic denervation might also be an alternative therapeutic option. Early identification and risk stratification is of major importance in patients with CPVT. The aim of the present review is to present the arrhythmogenic mechanisms of the disease, the current therapies applied and potential future perspectives.
- Published
- 2019