Cardiac amyloidosis: A practical approach to diagnosis and management

Prashant Kapoor, Thenappan Thenappan, Ekta Singh, Shaji Kumar, Philip R. Greipp

Research output: Contribution to journalReview article

71 Scopus citations

Abstract

Cardiac amyloidosis, the primary determinant of prognosis in systemic amyloidoses, is characterized by infiltration of myocardium by amyloid protein resulting in cardiomyopathy and conduction disturbances. Cardiac involvement is primarily encountered in immunoglobulin (AL) and transthyretin-associated (hereditary/familial and senile) amyloidoses. Although the latter variants could be indolent, untreated AL amyloidosis with clinical cardiac involvement is a rapidly fatal disease. The management decisions of cardiac amyloidosis are based on the underlying cause. Although treatment of senile systemic amyloidosis is largely supportive, the therapeutic approaches for AL amyloidosis include chemotherapy, autologous stem cell transplantation, and, rarely, cardiac transplantation. The familial variant is potentially curable with a liver ± cardiac transplantation. This narrative review outlines a practical approach to these challenging diagnoses in the face of rapidly evolving management strategies.

Original languageEnglish (US)
Pages (from-to)1006-1015
Number of pages10
JournalAmerican Journal of Medicine
Volume124
Issue number11
DOIs
StatePublished - Nov 1 2011

Keywords

  • AL
  • Amyloidosis
  • Cardiomyopathy
  • Familial
  • Heart failure
  • Hereditary
  • Light chain
  • Primary
  • Senile

ASJC Scopus subject areas

  • Medicine(all)

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