title: Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia.
abstract: BACKGROUND
  Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia syndrome, characterised by polymorphic ventricular tachycardia induced by adrenergic stress. CPVT can be caused by mutations the cardiac ryanodine receptor gene (RYR2) or mutations in the cardiac calsequestrin gene CASQ2. Structural heart disease is usually absent and the baseline ECG is usually normal. Patients with CPVT often present with exercise- or emotion induced syncope, the first presentation can also be sudden cardiac death.
  
  
  RESULTS
  Besides removal of triggers treatment with beta blockers is currently a class I indication in clinically diagnosed patients. Beta blockage should be titrated up to an effective level. The addition of flecainide seems to be a promising approach in patients where arrhythmias are not completely suppressed by beta blockers. A cardioverter-defibrillator (ICD) or left cervical sympathetic denervation might be considered under special circumstances. Genetic counselling is recommended and all first degree relatives should be properly evaluated.
authors: The Royal Children's Hospital Melbourne, Australia. andreas.pflaumer@rch.org.au
authors: Andreas
authors: A
authors: Pflaumer
authors: Andrew M
authors: AM
authors: Davis
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D004247:DNA / Q000235:genetics; D017147:Defibrillators, Implantable; D003937:Diagnosis, Differential; D004562:Electrocardiography / Q000379:methods*; D020022:Genetic Predisposition to Disease; D006801:Humans; D009154:Mutation; D017410:Practice Guidelines as Topic; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D000319:Adrenergic beta-Antagonists; D019837:Ryanodine Receptor Calcium Release Channel; D004247:DNA