Cardiomyopathy in the Kearns-Sayre syndrome. Issue 4 (April 1988)

• Record Type: Journal Article
• Authors: Channer, K S; Channer, J L; Campbell, M J; Rees, J R
• Is Part Of: Heart. Volume 59:Issue 4(1988)
• Page Start: 486
• Page End: 490
• Publication Date: 1988-04
• Subjects: Heart -- Diseases -- Treatment -- Periodicals; Cardiology -- Periodicals; 616.12
• DOI: 10.1136/hrt.59.4.486
• Languages: English
• ISSNs: 1355-6037

Abstract

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.