Document Detail

Infantile histiocytoid cardiomyopathy: three cases and literature review.
MedLine Citation:
PMID:  7942464     Owner:  NLM     Status:  MEDLINE    
A review is presented of the clinical and cardiac morphologic findings in 50 previously reported and 3 new patients with histiocytoid cardiomyopathy. This disorder occurs in infants and small children and is characterized clinically by severe and often fatal arrhythmias and morphologically by focal collections of altered myocytes that are roundshaped and resemble histiocytes. Sixteen patients had yellowish nodules on the endocardium, epicardium, and/or valves; the other 37 had foci of abnormal myocytes throughout the myocardium. These cells were remarkably similar in all patients and had poorly developed or absent intercellular junctions, few or no contractile elements, and markedly increased numbers of mitochondria, which imparted a granular or vacuolated appearance to the cytoplasm. There was a high prevalence of anomalies involving the nervous system and eyes and of oncocytic cells in various glands. Evidence is presented to exclude the possibilities that the disorder represents a developmental anomaly of the atrioventricular conduction system, a multifocal tumor of Purkinje cells, a developmental arrest of cardiac myocytes, and a diffuse type of mitochondrial cardiomyopathy. Histiocytoid cardiomyopathy is considered to be the result of hamartoma-like aggregations of cardiac myocytes with features similar to those of oncocytes. This syndrome is likely caused by prenatal myocardial or systemic (viral?) injury. Surgical excision of nodules of histiocytoid cells can result in clinical remission.
V Malhotra; V J Ferrans; R Virmani
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  American heart journal     Volume:  128     ISSN:  0002-8703     ISO Abbreviation:  Am. Heart J.     Publication Date:  1994 Nov 
Date Detail:
Created Date:  1994-11-18     Completed Date:  1994-11-18     Revised Date:  2006-02-27    
Medline Journal Info:
Nlm Unique ID:  0370465     Medline TA:  Am Heart J     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1009-21     Citation Subset:  AIM; IM    
Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC.
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MeSH Terms
Cardiomyopathies / epidemiology,  pathology*
Child, Preschool
Heart Conduction System / pathology*
Heart Defects, Congenital / epidemiology
Histiocytes / pathology*
Infant, Newborn
Myocardium / pathology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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