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Polymorphic and polytopic ventricular extrasystoles

MedGen UID:
870550
Concept ID:
C4024998
Disease or Syndrome
HPO: HP:0006696

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolymorphic and polytopic ventricular extrasystoles

Conditions with this feature

Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome
MedGen UID:
1781114
Concept ID:
C5542154
Disease or Syndrome
Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome (VACRDS) is characterized by syncope, cardiac arrest, and/or sudden unexpected death. Polymorphic ventricular tachycardia and ventricular fibrillation have been documented in these patients. Symptoms generally occur with physical activity or emotional stress, but unlike typical catecholaminergic ventricular tachycardia (CPVT), arrhythmias are not reproducible on exercise stress testing or adrenaline challenge (Sun et al., 2021). Mutation in the RYR2 gene also causes catecholaminergic polymorphic ventricular tachycardia-1 (CPVT1; 604772).

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