Document Detail

Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta, and its branches.
MedLine Citation:
PMID:  11343995     Owner:  NLM     Status:  MEDLINE    
We report an unusual case of "arrhythmic" sudden cardiac death in a 3-year-old child who died of ischemic myocardial lesions as a result of intimal fibroplasia of the coronary arteries. Also affected were the aorta and its major branches, whereas renal and mesenteric arteries, celiac trunk, and systemic veins were normal. Histopathologic examination showed severe concentric thickening of intima because of a proliferation of spindle-shaped cells (mesenchymal cells) set in an abundant extracellular matrix. In some vascular segments the intima was densely fibrotic and hyalinized. No significant inflammation, foam cells, cholesterol clefts, or other evidence of atheroma were present. The intimal lesions did not involve the media and/or the adventitia. Immunohistochemical staining of intima showed the proliferating mesenchimal cells to be myofibroblastic. Reactions for vimentin and smooth muscle actin were positive, while those for desmin, myosin, CD34, and Factor VIII were negative.
E Maresi; G Becchina; G Ottoveggio; E Orlando; R Midulla; R Passantino
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology     Volume:  10     ISSN:  1054-8807     ISO Abbreviation:  Cardiovasc. Pathol.     Publication Date:    2001 Jan-Feb
Date Detail:
Created Date:  2001-05-09     Completed Date:  2001-07-05     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9212060     Medline TA:  Cardiovasc Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  43-8     Citation Subset:  IM    
Department of Pathology, University of Palermo, Via Gustavo Rocella 61, 90128 Palermo, Italy.
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MeSH Terms
Actins / analysis
Aorta / pathology*
Arrhythmias, Cardiac / complications,  pathology*
Child, Preschool
Coronary Vessels / pathology*
Death, Sudden, Cardiac / etiology,  pathology*
Fibromuscular Dysplasia / complications,  pathology*
Heart Ventricles / pathology
Myocardium / pathology
Polyps / pathology
Tunica Intima / chemistry,  pathology*
Vimentin / analysis
Reg. No./Substance:
0/Actins; 0/Vimentin

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

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