Chronotropic incompetence as a risk predictor in children and young adults with catecholaminergic polymorphic ventricular tachycardia

J Cardiovasc Electrophysiol. 2019 Oct;30(10):1923-1929. doi: 10.1111/jce.14043. Epub 2019 Jul 11.

Abstract

Introduction: Risk stratification tools for catecholaminergic polymorphic ventricular tachycardia (CPVT) are limited. The exercise stress test (EST) is the most important diagnostic and prognostic test. We aimed to determine whether heart rate (HR) and blood pressure (BP) response during EST were associated with the risk of arrhythmias.

Materials and methods: We studied the association between HR and BP response and ventricular arrhythmia burden on EST in 20 CPVT patients. HR reserve values <80% and ≤62% were used to define chronotropic incompetence (CI) off and on therapy, respectively. Symptoms and ventricular arrhythmia score (VAS) in all patients with respect to CI and BP during index EST off therapy and on maximal therapy were compared.

Results: CI in CPVT patients off therapy was associated with a worse VAS during EST (P = .046). Patients with CI also more frequently presented with syncope and/or cardiac arrest compared to patients with a normal chronotropic response (P = .008). Once on therapy, patients with CI had similar VAS compared to patients without CI (P = .50), suggesting that treatment attenuates risk related to CI. Patients with CI also had a lower peak systolic BP (P = .041) which persisted on maximal therapy (P = .033).

Conclusion: Untreated CPVT patients with CI have more ventricular arrhythmias than those without CI. This may serve as a simple disease prognosticator that can be modified by antiarrhythmic therapy. A mechanistic link between CI and arrhythmia susceptibility remains unknown. Larger studies are needed to confirm and establish the mechanism of these findings.

Keywords: CPVT; chronotropic incompetence; exercise stress test; heart rate; ventricular arrhythmia.

Publication types

  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / therapeutic use
  • Age Factors
  • Anti-Arrhythmia Agents / therapeutic use
  • Blood Pressure* / drug effects
  • British Columbia
  • Child
  • Death, Sudden, Cardiac / prevention & control
  • Electrocardiography*
  • Exercise Test*
  • Female
  • Heart Rate* / drug effects
  • Humans
  • Male
  • Pilot Projects
  • Predictive Value of Tests
  • Prognosis
  • Registries
  • Retrospective Studies
  • Risk Factors
  • Tachycardia, Ventricular / diagnosis*
  • Tachycardia, Ventricular / mortality
  • Tachycardia, Ventricular / physiopathology
  • Tachycardia, Ventricular / therapy
  • Tennessee
  • Time Factors
  • Young Adult

Substances

  • Adrenergic beta-Antagonists
  • Anti-Arrhythmia Agents

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia