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Sinus Dysrhythmia in Kearns‐Sayre Syndrome
Author(s) -
ULICNY KARL S.,
DETTERBECK FRANK C.,
HALL COLIN D.
Publication year - 1994
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.1994.tb01445.x
Subject(s) - medicine , kearns–sayre syndrome , cardiology , biochemistry , chemistry , mitochondrial dna , gene
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.