Primary cardiac amyloidosis with 20-year survival

Cardiovasc Pathol. Nov-Dec 2006;15(6):331-5. doi: 10.1016/j.carpath.2006.06.002.

Abstract

Background: The natural history of primary amyloidosis is poor, and for patients with symptomatic cardiac involvement, survival is generally less than 6 months. Even among treated patients with amyloid heart disease, survival beyond 5 years is rare.

Case report: We report a patient with primary cardiac amyloidosis who is currently alive 20 years after his initial diagnosis. The extent and subtype of amyloid were documented by endomyocardial biopsy both at the time of initial diagnosis and 20 years later. To our knowledge, this is the longest survival ever reported for a patient with cardiac involvement by primary amyloidosis.

Conclusion: The remarkably long stabilization of amyloid deposition in this patient may be attributed to early diagnosis, early institution of therapy, and, possibly, favorable genetic factors.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amyloid / metabolism*
  • Amyloidosis / pathology*
  • Amyloidosis / physiopathology
  • Amyloidosis / therapy
  • Cardiomyopathies / pathology*
  • Cardiomyopathies / physiopathology
  • Cardiomyopathies / therapy
  • Colchicine / therapeutic use
  • Drug Therapy, Combination
  • Humans
  • Longevity
  • Male
  • Melphalan / therapeutic use
  • Myocardium / metabolism
  • Myocardium / pathology*
  • Prednisone / therapeutic use
  • Survival

Substances

  • Amyloid
  • Melphalan
  • Colchicine
  • Prednisone