Ventricular arrhythmia suppression with ivabradine in a patient with catecholaminergic polymorphic ventricular tachycardia refractory to nadolol, flecainide, and sympathectomy

Utkarsh Kohli, Zaid Aziz, Andrew D. Beaser, Hemal M. Nayak

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Conventional treatment strategies for catecholaminergic polymorphic ventricular tachycardia (CPVT) include avoidance of strenuous exercise and competitive sports, drugs such as ß-blockers and flecainide and, cervical sympathectomy. An implantable cardioverter-defibrillator (ICD) has been utilized if the response to these strategies is inadequate; however, ICD use in CPVT patients, in addition to usual complications, is associated with an increased risk of life-threatening electrical storm. Ivabradine is a selective inhibitor of hyperpolarization-activated cyclic nucleotide-gated potassium channel 4 generated funny current (If), which has been shown to be efficacious in suppression of inappropriate sinus tachycardia, junctional tachycardia, atrial tachycardia, and ventricular ectopy in humans. We report an 18-year-old male with a severe CPVT phenotype refractory to flecainide, nadolol, and sympathectomy who exhibited suppression of ventricular arrhythmias after initiation of ivabradine. These findings are of importance as ivabradine could be an important add-on therapy in CPVT patients who are drug refractory or are unable to continue conventional therapies at the recommended doses.

Original languageEnglish (US)
Pages (from-to)527-533
Number of pages7
JournalPACE - Pacing and Clinical Electrophysiology
Volume43
Issue number5
DOIs
StatePublished - May 1 2020
Externally publishedYes

Keywords

  • RYR2 exon 3 deletion
  • catecholaminergic polymorphic ventricular tachycardia
  • drug and sympathectomy refractory
  • ivabradine
  • malignant syncope

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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