What are the HRS/EHRA/APHRS guidelines for the diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT)?

Updated: Dec 05, 2017
  • Author: Steven J Compton, MD, FACC, FACP, FHRS; Chief Editor: Jeffrey N Rottman, MD  more...
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Answer

In its 2013 expert consensus statement on inherited primary arrhythmia syndromes, the Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society (HRS/EHRA/APHRS) indicated catecholaminergic polymorphic ventricular tachycardia (CPVT) can be diagnosed when any for the following criteria are met [84] :

  • Structurally normal heart, normal electrocardiogram (ECG), and unexplained exercise or catecholamine-induced bidirectional VT or polymorphic ventricular premature beats (VPBs) or VT in an individual younger than 40 years
  • Presence of a pathogenic mutation
  • Family history of CPVT with a normal heart, exercise-induced premature ventricular contractions or bidirectional/polymorphic VT
  • Structurally normal heart and coronary arteries, normal ECG, and unexplained exercise or catecholamine-induced bidirectional VT or polymorphic VPBs or VT in an individual older than 40 years

Management recommendations include [84] :

Class I

  • Limit or avoid competitive sports, strenuous exercise, and/or stressful environments
  • Beta blockers for all symptomatic patients
  • Placement of an implantable cardioverter-defibrillator for patients who experience cardiac arrest, recurrent syncope, or polymorphic/bidirectional VT despite optimal medical management, and/or left cardiac sympathetic denervation (LCSD)

Class IIa

  • Flecainide in addition to beta blockers in patients who experience recurrent syncope or polymorphic/bidirectional VT while on beta blockers
  • Beta blockers for carriers of a pathogenic CPVT mutation without clinical manifestations (concealed mutation-positive patients).

Class IIb

  • Consider LCSD in patients who experience recurrent syncope or polymorphic/bidirectional VT/several appropriate ICD shocks while on beta blockers and in patients for whom beta blockers are contraindicated.

Class III

  • ICD is not indicated as a stand-alone therapy in an asymptomatic CPVT patients. 
  • Programmed electrical stimulation is not indicated.

In general, the 2015 ESC guidelines concur with the recommendations above as well as include the following additional guidance [82] :

  • Flecainide should be considered in addition to beta blockers in patients with an ICD to reduce appropriate ICD shocks. (Class IIa)

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