Catecholaminergic polymorphic ventricular tachycardia

Chan W. Kim, Wilbert S. Aronow, Tanya Dutta, Daniel Frenkel, William H. Frishman

Research output: Contribution to journalReview articlepeer-review

5 Scopus citations

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare congenital arrhythmogenic disorder induced by physical or emotional stress. It mainly affects children and younger adults and is characterized by rapid polymorphic and bidirectional ventricular tachycardia. Symptoms can include dizziness, palpitations, and presyncope, which may progress to syncope, hypotonia, convulsive movements, and sudden cardiac death. CPVT is the result of perturbations in Ca2+ ion handling in the sarcoplasmic reticulum of cardiac myocytes. Mutations in the cardiac ryanodine receptor gene and the calsequestrin isoform 2 gene are most commonly seen in familial CPVT patients. Under catecholaminergic stimulation, either mutation can result in an excess Ca2+ load during diastole resulting in delayed after depolarization and subsequent arrhythmogenesis. The current first-line treatment for CPVT is β-blocker therapy. Other therapeutic interventions that can be used in conjunction with β-blockers include moderate exercise training, flecainide, left cardiac sympathetic denervation, and implantable cardioverter-defibrillators. Several potential therapeutic interventions, including verapamil, dantrolene, JTV519, and gene therapy, are also discussed.

Original languageEnglish
Pages (from-to)325-331
Number of pages7
JournalCardiology in Review
DOIs
StateAccepted/In press - 2020
Externally publishedYes

Keywords

  • Beta-blockers
  • Catecholaminergic polymorphic ventricular tachycardia
  • Flecainide
  • Implantable cardioverter-defibrillators
  • Left cardiac sympathetic denervation

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