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隐匿性心脏淀粉样变性的临床问题。法医学意义。

The clinical problem of occult cardiac amyloidosis. Forensic implications.

作者信息

Petersen E C, Engel J A, Radio S J, Canfield T M, McManus B M

机构信息

Cardiovascular Registry, University of Nebraska Medical Center, Omaha 68198-6495.

出版信息

Am J Forensic Med Pathol. 1992 Sep;13(3):225-9. doi: 10.1097/00000433-199209000-00012.

DOI:10.1097/00000433-199209000-00012
PMID:1476128
Abstract

A 68-year-old man with known coronary heart disease experienced rapidly progressive cardiac dysfunction and was found to have occult cardiac amyloidosis at autopsy. The amyloidosis was undiagnosed during life and initially at autopsy. Marked diffuse involvement of the intramural coronary arteries by amyloid deposits resulted in severe luminal compromise of numerous medium and small vessels. The myocardium proper was virtually spared from amyloid deposits. Amyloid-related coronary narrowing contributed to cardiac ischemia and sudden death. The significance of amyloid coronary disease in this patient relates primarily to the difficulty in considering the diagnosis when other reasons for cardiac signs and symptoms preexist. Also, the adverse effects of amyloid coronary disease may be profound without direct myocardial involvement.

摘要

一名患有冠心病的68岁男性出现快速进展的心脏功能障碍,尸检时发现隐匿性心脏淀粉样变性。淀粉样变性在生前及尸检初期均未被诊断出来。淀粉样沉积物显著弥漫性累及壁内冠状动脉,导致众多中小血管严重管腔狭窄。心肌本身几乎未受淀粉样沉积物影响。淀粉样变性相关的冠状动脉狭窄导致心脏缺血和猝死。该患者中淀粉样变性冠状动脉疾病的重要性主要在于当存在心脏体征和症状的其他原因时难以考虑到该诊断。此外,淀粉样变性冠状动脉疾病在无直接心肌受累的情况下可能产生严重不良影响。

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