TITLE

Myocardial Ultrastructure in Kearns-Sayre Syndrome

AUTHOR(S)
Nakano, Takeshi; Imanaka, Kyoko; Uchida, Hideki; Isaka, Naoki; Takezawa, Hideo
PUB. DATE
January 1987
SOURCE
Angiology;Jan1987 Part 1, Vol. 38 Issue 1, p28
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Myocardial ultrastructural changes are described in a twenty-five-year-old female with typical Kearns-Sayre syndrome. The electrocardiogram revealed complete atrioventricular block with an occasional torsades de pointes type of ventricular tachycardia. His bundle recording demonstrated HV block with normal AH interval. An endomyocardial biopsy showed "ragged-red fibers" by the modified Gomori trichrome stain, and on electron microscopy there were increased numbers of morphologically abnormal mitochondria: ie, giant mitochondria, a whorled pattern of the cristae, and electron-dense substances, and paracrystalline inclusions in the mitochondria. The patient has been well since pacemaker implantation, but she may develop a clinically overt cardiomyopathy due to progression of myocardial involvement.
ACCESSION #
16478564

 

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