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, and complete heart block, as well as non-specific 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.
Ulicny
K S
KS
Department of Surgery, University of North Carolina School of Medicine, Chapel Hill.
Detterbeck
F C
FC
Hall
C D
CD
D000293:Adolescent; D001146:Arrhythmia, Sinus / Q000150:complications* / Q000503:physiopathology; D004562:Electrocardiography; D005500:Follow-Up Studies; D006323:Heart Arrest / Q000150:complications* / Q000503:physiopathology; D006327:Heart Block / Q000150:complications / Q000503:physiopathology; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000150:complications*; D008297:Male