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Cardiac Amyloidosis

  • Carmela D. TanEmail author
  • E. Rene Rodriguez
Part of the Current Clinical Pathology book series (CCPATH)

Abstract

Involvement of the cardiovascular system by amyloid deposits can be a localized phenomenon or part of a systemic disease. Isolated atrial, valvular, and aortic amyloidoses are in most instances asymptomatic and incidental findings in surgical specimens. Symptomatic cardiac amyloidosis is due to deposition of immunoglobulin light chains or transthyretin in the majority of cases. Except for familial transthyretin-associated amyloidosis, the heart is rarely involved in other hereditary types of amyloidosis. Cardiac amyloidosis is an underdiagnosed cause of heart failure. While electrocardiographic, echocardiographic, and magnetic resonance imaging findings could be highly suspicious for amyloidosis, definitive diagnosis and amyloid typing require an endomyocardial biopsy. The typical gross and microscopic features of cardiac amyloidosis are presented, as well as potential pitfalls and caveats in amyloid typing. Identifying the correct amyloid protein type is important for therapeutic management and prognostication. Early diagnosis remains a challenging problem. The prognosis of patients with cardiac amyloidosis is still poor because of advanced stage at the time of diagnosis and limited effectiveness of current treatment modalities.

Keywords

Light chain amyloidosis Transthyretin amyloidosis Atrial Valvular Aortic Senile systemic amyloidosis Light chain deposition disease Atrial natriuretic factor Apolipoprotein A1 Endomyocardial biopsy 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  1. 1.Department of PathologyCleveland Clinic Lerner College of Medicine of Case Western Reserve UniversityClevelandUSA

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