Catecholaminergic polymorphic ventricular tachycardia: a current overview.
Catecholaminergic polymorphic ventricular tachycardia occurs in healthy children and young adults causing syncope and sudden cardiac death. This is a familial disease, which affect de novo mutation in 50% of the cases. At least two causative genes have been described to be localized in the chromosome 1; mutation of the ryanodine receptor gene and calsequestrin gene. The classical clinical presentation is syncope triggered by exercise and emotion in children and adolescents with no structural heart disease. Polymorphic ventricular tachycardia during treadmill testing, or after isoproterenol infusion, is the most common feature. Therapeutic options include, beta-blockers, calcium-channel blockers and, an implantable cardioverter defibrillator is indicated in high-risk patients. Risk stratification of this disease is very challenging, since some risk factors proved to be useful in some series but not in others. However, family history of sudden cardiac death and symptoms initiated in very young children are important predictors.
Leite
Luiz R
LR
Grupo de Estudos em Arritmias Cardíacas/Fibrilação Atrial, Brasília DF, SMDB Conj., 16 Lote 5 Casa 1, Brasília 71680-160, DF, Brazil. leite.luiz@brturbo.com.br
Henz
Benhur D
BD
Macedo
Paula G
PG
Santos
Simone N
SN
Barreto
José R
JR
Zanatta
André
A
Fenelon
Guilherme
G
Cruz Filho
Fernando E S
FE
D000319:Adrenergic beta-Antagonists / Q000008:administration & dosage / Q000009:adverse effects*; D002648:Child; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D015716:Electrocardiography, Ambulatory; D017809:Fatal Outcome; D005260:Female; D006339:Heart Rate / Q000187:drug effects; D006801:Humans; D011433:Propranolol / Q000008:administration & dosage / Q000009:adverse effects*; D017180:Tachycardia, Ventricular / Q000139:chemically induced* / Q000175:diagnosis / Q000503:physiopathology
D000319:Adrenergic beta-Antagonists; D011433:Propranolol