Oncocytic cardiomyopathy syndrome.

Abstract

The case presented is the first report of oncocytic cardiomyopathy associated with congenital heart disease. The clinical presentation was typical of hypoplastic left heart syndrome, and the oncocytic cardiomyopathy was an unexpected finding at autopsy. The associated lesions of oncocytic metaplasia in thyroid and pituitary gland have been previously reported. Maternal serology and viral cultures from the infant were negative, indicating that the oncocytic lesions were not secondary to congenital viral infection. We support the theory that oncocytic cardiomyopathy is a hamartomatous lesion, and propose as a paradigm the association between cardiac rhabdomyoma and tuberous sclerosis. Careful follow-up of surgically "cured" oncocytic cardiomyopathy should uncover oncocytomas in other organs later in life.

• Authors: R A Franciosi, A Singh
• Journal: Human pathology (Hum Pathol) Vol. 19 Issue 11 Pg. 1361-2 (Nov 1988) ISSN: 0046-8177 [Print] United States
• PMID: 3181957 (Publication Type: Case Reports, Journal Article)

Topics

• Female
• Heart Defects, Congenital (complications)
• Heart Neoplasms (complications)
• Humans
• Infant, Newborn
• Syndrome