¹èÀºÁ¤(Bae Eun-Jung) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ¼Ò¾Æ°úÇб³½Ç
±Çº¸»ó(Kwon Bo-Sang) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ¼Ò¾Æ°úÇб³½Ç
Abstract
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly malignant form of inherited arrhythmogenic disorder characterized by episodes of syncope or sudden death in response to physiological or emotional stress. As genetic backgrounds for the autosomal-dominant and recessive forms of CPVT, mutations in genes encoding cardiac ryanodine type 2 receptor (RYR2) and calsequestrin 2 (CASQ2) were found. The diagnosis of CPVT based on the stress induced reproducible polymorphic ventricular tachycardia in patients with normal resting electrocardiogram without structural cardiac abnormalities. Charateristrically many CPVT patients was misdiagnosed as epilepsy in children and young adult. The medical treatment with beta blocker is the first line therapy. In addition, an implantable cardioverter defibrillator implantation can be also helpful. However such standard therapy is occasionally ineffective and overall mortality rate reaches 30~50%.
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catecholaminergic polymorphic ventricular tachycardia, syncope, sudden death
KMID :
1011920090100020028
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À¯È¿¼º°á°ú(Recomendation)
The medical treatment with beta blocker is the first line therapy; An implantable cardioverter defibrillator implantation can be also helpful.