Catecholaminergic polymorphic ventricular tachycardia: A paradigm to understand mechanisms of arrhythmias associated to impaired Ca2+ regulation

被引：76

作者：

Cerrone, Marina ^{[2
]}

Napolitano, Carlo ^{[1
,2
]}

Priori, Silvia G. ^{[1
,2
,3
]}

机构：

[1] Fdn S Maugeri IRCCS, Mol Cardiol Labs, I-27100 Pavia, Italy

[2] NYU, Sch Med, Leon H Charney Div Cardiol, New York, NY USA

[3] Univ Pavia, Dept Cardiol, I-27100 Pavia, Italy

来源：

HEART RHYTHM | 2009年 / 6卷 / 11期

关键词：

CARDIAC RYANODINE RECEPTOR; IN MOUSE MODEL; CALCIUM-RELEASE; SUDDEN-DEATH; RYR2; MUTATIONS; MOLECULAR CHARACTERIZATION; DEFECTIVE REGULATION; EXERCISE; CASQ2; CALSEQUESTRIN;

D O I：

10.1016/j.hrthm.2009.06.033

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

In the 8 years since the discovery of the genetic bases of catecholaminergic polymorphic ventricular tachycardia (CPVT), we have witnessed a remarkable improvement of knowledge on arrhythmogenic mechanisms involving disruption of cardiac Ca2+ homeostasis. Studies on the consequences of RyR2 and CASQ2 mutations in cellular systems and mouse models have shed new Light on pathways that are also implicated in arrhythmias occurring in highly prevalent diseases, such as heart failure. This research track has also led to the identification of therapeutic targets of potential clinical impact to abate the burden of sudden death in CPVT. Here, we review the current knowledge on the pathophysiology of CPVT also highlighting the existing controversies and possible future development.

引用

页码：1652 / 1659

页数：8

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