Sinus Dysrhythmia in Kearns‐Sayre Syndrome

Kearns‐Sayre syndrome is the triad of progressive external ophthalmoplegia, pigmentary retinopathy, and complete AV block. The etiology is unknown, but is thought to be due to a mitochondrial DNA deletion. Reported electrocardiographic abnormalities include first‐degree AV block, fascicular blocks,...

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Bibliographic Details
Published in:Pacing and clinical electrophysiology 1994-05, Vol.17 (5), p.991-994
Main Authors: ULICNY, KARL S., DETTERBECK, FRANK C., HALL, COLIN D.
Format: Article
Language:English
Subjects:
Adolescent
Arrhythmia, Sinus - complications
Arrhythmia, Sinus - physiopathology
Electrocardiography
Follow-Up Studies
Heart Arrest - complications
Heart Arrest - physiopathology
Heart Block - complications
Heart Block - physiopathology
Humans
Kearns-Sayre Syndrome - complications
Kearns‐Sayre syndrome
Male
mitochondrial cytopathies
sinus dysrhythmia
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Summary:Kearns‐Sayre syndrome is the triad of progressive external ophthalmoplegia, pigmentary retinopathy, and complete AV block. The etiology is unknown, but is thought to be due to a mitochondrial DNA deletion. Reported electrocardiographic abnormalities include first‐degree AV block, fascicular blocks, and complete heart block, as well as nonspecific S‐T segment changes and T wave abnormalities, but has not included sinus node dysfunction. We report a case with episodes of sinus arrest in an asymptomatic patient with Kearns‐Sayre syndrome resulting in pauses lasting up to 6 seconds.
ISSN:0147-8389
1540-8159
DOI:10.1111/j.1540-8159.1994.tb01445.x