Key result
Kearns-Sayre syndrome triggers torsade de pointes cardiac arrest despite pacemaker therapy.
Why the study?
Complete heart block and ventricular tachycardia are implicated mechanisms of sudden death in Kearns-Sayre syndrome, but management strategies require clarification.
Case Report (n=1)
Highlights the risk of torsade de pointes and sudden cardiac death in Kearns-Sayre syndrome, suggesting a potential need for ICDs rather than just pacemakers.
A 47-year-old woman with Kearns-Sayre syndrome (KSS) and an implanted pacemaker for complete heart block was admitted to the intensive care unit following a cardiac arrest due to ventricular tachycardia (torsade de pointes) in the setting of QT prolongation. Complete heart blocks and ventricular tachycardia are implicated as mechanisms of sudden deaths in KSS; such patients may require pacemaker implantation and implantation of an automatic implantable cardioverter-defibrillator.
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Wilmin et al. (2012) conducted a case report in Kearns-Sayre syndrome (n=1). Kearns-Sayre syndrome with QT prolongation was evaluated on Cardiac arrest due to ventricular tachycardia (torsade de pointes). A 47-year-old woman with Kearns-Sayre syndrome and an implanted pacemaker experienced cardiac arrest due to torsade de pointes in the setting of QT prolongation.
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