Cardiomyopathy in the Kearns-Sayre syndrome.

Abstract

The Kearns-Sayre syndrome is a mitochondrial myopathy characterised by ptosis, chronic progressive external ophthalmoplegia, abnormal retinal pigmentation, and cardiac conduction defects. A unique case is reported in which there was rapid development of progressive congestive cardiac failure that required cardiac transplantation. A review of published reports of mitochondrial myopathy shows that a minority of cases (less than 20%) have cardiac involvement. This had previously been limited to abnormalities of cardiac conduction with progressive heart block. Myocardial biopsy has, however, shown ultrastructural evidence of a generalised mitochondrial disorder which hitherto has not been associated with a functional deficit.

• Authors: K S Channer, J L Channer, M J Campbell, J R Rees
• Journal: British heart journal (Br Heart J) Vol. 59 Issue 4 Pg. 486-90 (Apr 1988) ISSN: 0007-0769 [Print] England
• PMID: 3370184 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Cardiomyopathy, Dilated (etiology, pathology)
• Eye Movements
• Humans
• Kearns-Sayre Syndrome (complications)
• Male
• Myocardium (ultrastructure)
• Ophthalmoplegia (complications)