Alarming atrioventricular block and mitral valve prolapse in the Kearns-Sayre syndrome (Journal article)

Katsanos, K. H./ Pappas, C. J./ Patsouras, D./ Michalis, L. K./ Kitsios, G./ Elisaf, M. S./ Tsianos, E. V.

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dc.contributor.authorKatsanos, K. H.en
dc.contributor.authorPappas, C. J.en
dc.contributor.authorPatsouras, D.en
dc.contributor.authorMichalis, L. K.en
dc.contributor.authorKitsios, G.en
dc.contributor.authorElisaf, M. S.en
dc.contributor.authorTsianos, E. V.en
dc.rightsDefault Licence-
dc.subjectEchocardiography, Doppleren
dc.subjectFatal Outcomeen
dc.subjectHeart Arresten
dc.subjectHeart Block/diagnosis/*etiologyen
dc.subjectKearns-Sayre Syndrome/*complications/diagnosisen
dc.subjectMitral Valve Prolapse/diagnosis/*etiologyen
dc.titleAlarming atrioventricular block and mitral valve prolapse in the Kearns-Sayre syndromeen
heal.type.enJournal articleen
heal.type.elΆρθρο Περιοδικούel
heal.recordProviderΠανεπιστήμιο Ιωαννίνων. Σχολή Επιστημών Υγείας. Τμήμα Ιατρικήςel
heal.abstractKearns-Sayre syndrome (KSS) is a multisystem mitochondrial disorder characterized by the invariant triad: onset before 20, progressive external ophthalmoplegia and pigmentary retinal degeneration, plus at least one of the following: complete (or not) heart block, cereberal dysfunction and CSF protein above 100 mg/dl. Autopsies from patients with KSS revealed widespread tissue distribution mtDNA deletions. These deletions result in significantly lower activities of the enzymes of the respiratory chain. The same deletion of mitochondrial DNA present in skeletal muscle is found in myocardial tissue. An 18-year-old girl diagnosed with the KSS was admitted to our hospital because of an upper respiratory tract infection and dysphagia. ECG showed cardiac conduction defects. The patient had no history of syncope. At her surface ECG there was a complete RBBB (QRS duration approximately 130 ms), a clockwise rotation with an axis of approximately 90 degrees and a slight QT prolongation (420 ms). Echocardiography showed prolapse with thickening and degeneration of both mitral valve leaflets but without mitral regurgitation. The patient was started on a diet rich in potassium and pharmaceutical therapy with magnesium oxide (240 mg of elemental Mg p.o. per day), 1 g of calcium carbonate t.i.d., vitamin D (calcitriol 0.25 microg p.o. per day) and coenzyme Q(10) 100 mg daily and discharged 6 days later with slightly improved biochemical profile but apparent clinical improvement. Urgent pacemaker implantation was decided but unfortunately the patient died due to acute cardiac arrest 10 days later.en
heal.journalNameInt J Cardiolen
Appears in Collections:Άρθρα σε επιστημονικά περιοδικά ( Ανοικτά)

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