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catecholaminergic polymorphic ventricular tachycardia; stress-induced polymorphic ventricular tachycardia
Genetics:
- autosomal dominant form associated with defects in RYR2 (type1)
- autosomal recessive form associated with defects in CASQ2 (type 2)
- autosomal dominant form associated with defects in CALM1 (type4)
- may be associated with defects in CALM3
Clinical manifestations:
- syncope, seizures, or sudden death after physical activity or emotional stress
Special laboratory:
electrocardiogram
- stress-induced polymorphic ventricular tachycardia
Complications:
- polymorphic ventricular tachycardia may degenerate into cardiac arrest & cause sudden death
Management:
- beta-blocker
- implantable defibrillator
- abstinence from exercise [4]
General
genetic disease of the heart
polymorphic ventricular tachycardia
Database Correlations
OMIM correlations
References
- OMIM :accession 604772
- OMIM :accession 611938
- Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT1)
http://www.hfea.gov.uk/8364.html
- Medical Knowledge Self Assessment Program (MKSAP) 17,
American College of Physicians, Philadelphia 2015