title: Rapid development of life-threatening complete atrioventricular block in Kearns-Sayre syndrome.
abstract: Kearns-Sayre syndrome is a rare mitochondrial disorder with defined diagnostic criteria. Knowledge of these diagnostic criteria and early diagnosis are important to ensure periodic electrocardiograms for identification of cardiac conduction disorders, which are the most important prognostic factor of the disease. We report on a 9-year-old girl with rapid development of a life-threatening complete atrioventricular block within 10 months and discuss the importance and time interval of regular electrocardiograms. Our patient survived by placing a temporary transvenous pacemaker lead followed by permanent pacemaker implantation a few days later.
authors.affiliation: Department of Neonatology and Paediatric Intensive Care, University Hospital of Cologne, Children's Hospital, Kerpener Str. 62, 50937, Cologne, Germany. lars.welzing@uk-koeln.de
authors.forename: L
authors.initials: L
authors.lastname: Welzing
authors.forename: J C
authors.initials: JC
authors.lastname: von Kleist-Retzow
authors.forename: A
authors.initials: A
authors.lastname: Kribs
authors.forename: F
authors.initials: F
authors.lastname: Eifinger
authors.forename: C
authors.initials: C
authors.lastname: Huenseler
authors.forename: N
authors.initials: N
authors.lastname: Sreeram
mesh_terms: D054537:Atrioventricular Block / Q000209:etiology* / Q000628:therapy; D002648:Child; D018450:Disease Progression; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D010138:Pacemaker, Artificial; D013997:Time Factors