Non-ischemic scar underlines ventricular arrhythmias in Kearns-Sayre syndrome

  • Stefano Figliozzi Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Alessandro Zorzi | alessandrozorzi@gmail.com Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Martina Perazzolo Marra Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Alessandro Ruocco Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Sabino Iliceto Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Domenico Corrado Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.
  • Chiara Calore Department of Cardiac, Thoracic, Vascular and Public Health Sciences, University of Padova, Italy.

Abstract

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which cardiac involvement has been associated with poor prognosis. Although the most common clinical manifestation is progressive conduction system impairment, patients can suffer from ventricular arrhythmias. Yet, they show a high prevalence of sudden cardiac death, whose etiopathological mechanism is not completely understood. Cardiac magnetic resonance is a rising tool to detect subclinical heart involvement in many heart diseases and was recently able to detect nonischemic scar, which is an arrhythmogenic substrate, in patients affected by KSS.

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Published
2019-07-29
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Issue
Section
Clinical and Experimental Cases/Hypothesis
Keywords:
Mitochondrial cardiomyopathy, Kearns-Sayre syndrome, ventricular arrhythmias, imaging, cardiac magnetic resonance, non-ischemic scar.
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How to Cite
Figliozzi, S., Zorzi, A., Perazzolo Marra, M., Ruocco, A., Iliceto, S., Corrado, D., & Calore, C. (2019). Non-ischemic scar underlines ventricular arrhythmias in Kearns-Sayre syndrome. Cardiogenetics, 9(1). https://doi.org/10.4081/cardiogenetics.2019.8194