Cardiac Arrest in Kearns–Sayre Syndrome

Abstract

The prognosis of progressive ophthalmoplegia in patients with large-scale mitochondrial DNA deletions is highly variable and almost unpredictable.

Cardiac disease in Kearns-Sayre syndrome requires comprehensive management

“…5,6 We also do not agree with the management of the presented patient. Since patients with Kearns-Sayre syndrome not only develop atrioventricular block but also ventricular arrhythmias 5 and experience sudden cardiac death, 7 we should be informed about the results of long-term electrocardiography recordings by means of a reveal recorder. Kearns-Sayre syndrome patients may not only require implantation of a pacemaker but rather implantation of a loop recorder or an implantable cardioverter defibrillator.…”

“…We do not agree with the statement in the introduction that cardiac complications of Kearns-Sayre syndrome only include left anterior fascicular block, right bundle branch block, and complete atrioventricular block. 1 Cardiac involvement in Kearns-Sayre syndrome is variable and may also include dilated cardiomyopathy 2 requiring a ventricular assist device, 3 bradycardiarelated polymorphic ventricular tachycardia, 4 myocardial scarring, 4 or ventricular arrhythmias, including QT-prolongation, 5 ventricular tachycardia, 5 or torsades des pointes. 5,6 We also do not agree with the management of the presented patient.…”

“…1 Cardiac involvement in Kearns-Sayre syndrome is variable and may also include dilated cardiomyopathy 2 requiring a ventricular assist device, 3 bradycardiarelated polymorphic ventricular tachycardia, 4 myocardial scarring, 4 or ventricular arrhythmias, including QT-prolongation, 5 ventricular tachycardia, 5 or torsades des pointes. 5,6 We also do not agree with the management of the presented patient. Since patients with Kearns-Sayre syndrome not only develop atrioventricular block but also ventricular arrhythmias 5 and experience sudden cardiac death, 7 we should be informed about the results of long-term electrocardiography recordings by means of a reveal recorder.…”

Aborted Sudden Cardiac Death and a Mother with Suspected Metabolic Myopathy

“…Aborted SCD or SCD have been repeatedly reported in patients with MM 7–10. SCD in these patients occurred with or without previous cardiac disease.…”

“…SCD in these patients occurred with or without previous cardiac disease. The metabolic defect in these patients may reside within the oxidative,7 fatty acid,8 or glycogen metabolism 9. Previous studies have already shown that MM might be associated with pituitary adenoma 11.…”