Senile Cardiac Amyloidosis with Myocardial Dysfunction

Lyle J. Olson, Morie A. Gertz, William D. Edwards, Chin Yang li, Patricia A. Pellikka, David R. Holmes, A. jamil Tajik, Robert A. Kyle

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Abstract

Senile cardiac amyloid discovered at autopsy is usually regarded as an incidental finding. However, in immunohistochemical studies of autopsy material, three distinct forms of senile cardiovascular amyloid have been characterized, including a systemic form that diffusely infiltrates the cardiac ventricles. The systemic form can be identified immunohistochemically with use of antiserum to human prealbumin. We diagnosed senile systemic amyloidosis causing cardiac dysfunction in five men (57 to 72 years old) by using antiserum to prealbumin in myocardial biopsy tissue. Clinically, the five patients were indistinguishable from patients with nonsecretory immunoglobulin-derived primary amyloidosis with cardiac involvement; only immunohistochemical staining of myocardial tissue distinguished between the two entities. This distinction is important, because the treatment and prognosis of the two disorders are different. We recommend immunohistochemical staining of myocardial tissue for prealbumin in patients with biopsy-proved cardiac amyloid in whom no monoclonal immunoglobulin light chain is detectable in the serum or urine. (N Engl J Med 1987; 317:738–42.), SENILE cardiac amyloid is a frequent postmortem finding in patients more than 80 years of age and has generally been regarded as nonspecific and of no functional importance.1 In contrast, clinically important amyloid deposition in the heart has been thought to occur predominately in multiple myeloma and primary systemic amyloidosis and less often in the familial amyloidoses.2,3 In autopsy studies, however, senile cardiac amyloid has been retrospectively associated with antemortem congestive heart failure and cardiac arrhythmias.4 5 6 The senile cardiac amyloidoses are a heterogeneous group of disorders that cannot be distinguished by routine light microscopy or transmission electron microscopy. Three distinct…

Original languageEnglish (US)
Pages (from-to)738-742
Number of pages5
JournalNew England Journal of Medicine
Volume317
Issue number12
DOIs
StatePublished - Sep 17 1987

ASJC Scopus subject areas

  • Medicine(all)

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