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- W1991567266 abstract "Kearns–Sayre syndrome (KSS) is a mitochondrial disorder characterised by onset before the age of 20 years, progressive external ophthalmoplegia, and pigmentary retinopathy, accompanied by either cardiac conduction defects, elevated cerebrospinal fluid protein or cerebellar ataxia. 50% of patients with KSS develop cardiac complications. The most common cardiac manifestation is conduction disease which may progress to complete atrioventricular block or bradycardia-related polymorphic ventricular tachycardia (PMVT). The management of cardiac electrical disease associated with KSS and mitochondrial cytopathy is systematically reviewed including the case of a 23 year-old female patient with KSS who developed a constellation of cardiac arrhythmias including rapidly progressive conduction system disease and monomorphic ventricular tachycardia with myocardial scarring. The emerging role of cardiac magnetic resonance imaging (CMR) in detecting subclinical cardiac involvement is also highlighted. This review illustrates the need for cardiologists to be informed about this rare but emerging condition." @default.
- W1991567266 created "2016-06-24" @default.
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- W1991567266 date "2015-02-01" @default.
- W1991567266 modified "2023-10-16" @default.
- W1991567266 title "Systematic review of cardiac electrical disease in Kearns–Sayre syndrome and mitochondrial cytopathy" @default.
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- W1991567266 doi "https://doi.org/10.1016/j.ijcard.2014.12.038" @default.
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