Key result
Sustained polymorphic VT causes syncope without QT prolongation in Kearns-Sayre syndrome.
Why the study?
Cardiac dysfunction including sudden death in Kearns-Sayre syndrome patients involves conduction abnormalities, but characteristics of sustained polymorphic VT without QT prolongation or bradycardia are not well described.
Case Report (n=1)
Highlights the risk of sustained polymorphic VT without QT prolongation or bradycardia in Kearns-Sayre syndrome, suggesting a role for ICD implantation.
Polymorphic VT may cause syncope in Kearns-Sayre syndrome; hypothesis-generating for ICD use, should not yet change practice.
Cardiac dysfunction, including congestive heart failure and impulse conduction abnormalities, have been implicated in the sudden death of patients suffering from the Kearns-Sayre syndrome. This report describes a 29-year-old woman who suffered syncopal episodes due to sustained polymorphic VT. Sustained polymorphic VT was not associated with prolongation of the QT interval or bradycardia on the ECG. The implantation of a cardioverter defibrillator should be considered in this syndrome.
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Oginosawa et al. (2003) conducted a case report in Kearns-Sayre syndrome (n=1). Sustained polymorphic ventricular tachycardia was evaluated on Syncopal episodes. Sustained polymorphic ventricular tachycardia without QT prolongation or bradycardia caused syncopal episodes in a 29-year-old woman with Kearns-Sayre syndrome.
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