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Ventricular arrhythmias in Kearns-Sayre syndrome: A cohort study using the National Inpatient Sample database

Kyle Wiseman1, Dhairya Gor1, Ndausung Udongwo1

  • 1Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, New Jersey, USA.

Pacing and Clinical Electrophysiology : PACE
|October 8, 2022
PubMed

Insights

Patients with Kearns-Sayre syndrome (KSS) have a lower risk of ventricular arrhythmias (VA) than those with myotonic dystrophy (MD). Permanent pacemaker (PPM) use is underutilized in KSS patients with conduction abnormalities, despite its benefits.

Area of Science:

  • Cardiology
  • Genetics
  • Electrophysiology

Background:

  • Kearns-Sayre syndrome (KSS) is associated with cardiac conduction system degeneration, leading to complete heart block (CHB), ventricular arrhythmias (VA), and sudden cardiac death (SCD).
  • Permanent pacemaker (PPM) implantation is a potential preventive measure for SCD in KSS, but the efficacy of defibrillators and other SCD mechanisms require further investigation.

Purpose of the Study:

  • To investigate the risk of VA or dysrhythmic cardiac arrest (dCA) in patients with KSS.
  • To compare cardiac outcomes and device implantation strategies between KSS and myotonic dystrophy (MD) patients.

Main Methods:

  • Utilized the National Inpatient Sample (NIS) database from 2016-2019.
  • Compared outcomes of 640 KSS admissions to those with MD, focusing on VA/dCA incidence and device implantation.

Main Results:

  • KSS patients had a significantly lower incidence of VA or dCA compared to MD patients (2.3% vs. 4.5%, p=0.009).
  • Conduction abnormalities were linked to higher VA/dCA rates in both groups.
  • PPM (±defibrillator) was implanted in ~70% of KSS patients with conduction abnormalities at discharge, versus 35% in MD patients.

Conclusions:

  • PPM is underutilized in KSS and MD patients with conduction abnormalities, despite its effectiveness in preventing VA.
  • PPM alone may not fully prevent VA in MD; defibrillator capacity might be necessary.
Abstract

Keywords:
Kearns-Sayre syndromeconduction defectsimplantable cardioverter-defibrillatormyotonic dystrophypacemakerventricular tachycardia

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