Document Detail

Pathophysiology and management of syncope in Kearns-Sayre syndrome.
MedLine Citation:
PMID:  17086014     Owner:  NLM     Status:  MEDLINE    
A 20-year-old woman with known Kearns-Sayre syndrome was transferred to the emergency department due to syncopal episodes. The electrocardiogram on admission showed complete atrioventricular block. The diagnosis of mitochondrial encephalomyopathy was made when she was 14 years old. At the time of the initial diagnosis, she displayed a normal electrocardiogram pattern. At the age of 17, electrocardiogram recordings demonstrated right bundle branch block with left anterior fascicular block and a prolonged QTc interval of 485 milliseconds (Figure). She was taking coenzyme Q10, oral nicotinamide adenine dinucleotide (reduced), piribedil, amantadine, and primidone. Transthoracic echocardiography revealed normal wall motion of both ventricles and mitral valve prolapse without regurgitation. A permanent dual-chamber pacemaker was immediately implanted.
Konstantinos P Letsas; Michalis Efremidis; Loukas K Pappas; Gerasimos Gavrielatos; Antonios Sideris; Christos Charitos
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The American heart hospital journal     Volume:  4     ISSN:  1541-9215     ISO Abbreviation:  Am Heart Hosp J     Publication Date:  2006  
Date Detail:
Created Date:  2006-11-06     Completed Date:  2007-05-29     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  101156064     Medline TA:  Am Heart Hosp J     Country:  United States    
Other Details:
Languages:  eng     Pagination:  301-2     Citation Subset:  IM    
Second Department of Cardiology, Evangelismos General Hospital of Athens, Athens, Greece.
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MeSH Terms
Cardiac Pacing, Artificial
Heart Block / complications,  physiopathology,  therapy
Kearns-Sayre Syndrome / complications,  physiopathology*,  therapy*
Long QT Syndrome / complications,  physiopathology,  therapy
Mitral Valve Prolapse / physiopathology,  therapy
Pacemaker, Artificial*
Syncope / etiology,  physiopathology*,  therapy*

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