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CMAJ • September 2, 2003; 169 (5)
© 2003 Canadian Medical Association or its licensors

Letters
Correspondance

The heart of the matter

John P. Veinot

Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Ont.

Sandeep Arora and associates1 recommend extracardiac biopsy and other diagnostic modalities instead of heart biopsy for diagnosis of cardiac amyloidosis. However, endomyocardial biopsy remains an excellent method of demonstrating this problem, and false-negative results are uncommon in patients with heart failure.2

Immunohistochemical typing of the amyloid may be prognostic. Primary (amyloid light-chain [AL]) amyloidosis with resultant heart failure is associated with a poor prognosis, and up to 40% of such patients die of heart disease.3 In contrast, senile amyloidosis, which is common, is often uncomplicated, and treatment with cytotoxic agents may not be required.4,5

Algorithms have been proposed to diagnose amyloidosis.5,6 Depending on the amyloid type, the results of extracardiac staining may not accurately indicate the presence of cardiac amyloid.3 Furthermore, in a study of patients with AL-type amyloidosis who had positive results on endomyocardial biopsy, the extracardiac biopsy results were not always positive.3

In patients with severe heart failure, biopsy-proven extracardiac amyloid site, characteristic electrocardiographic findings and characteristic echocardiographic changes, most clinicians feel confident in attributing cardiac dysfunction to amyloidosis.3 However, doing so may underestimate or overestimate cardiac involvement, depending on the patient population. Endomyocardial biopsy may be the only way to diagnose amyloidosis if it is confined to the heart. Immunotyping of the biopsy specimen may add prognostic information. Heart biopsy is also useful in distinguishing restrictive myocardial abnormalities from constrictive pericardial disease.

John P. Veinot Pathology and Laboratory Medicine University of Ottawa Ottawa, Ont.

References

  1. Arora S, Arora A, Makkar RPS, Monga A. Stiff heart syndrome. CMAJ 2003;168(13):1690-1.[Free Full Text]
  2. Frenzel H, Schwartzkopff B, Kuhn H, Losse B, Thormann J, Hort W, et al. Cardiac amyloid deposits in endomyocardial biopsies: light microscopic, ultrastructural, and immunohistochemical studies. Am J Clin Pathol 1986;85:674-80.[Medline]
  3. Pellikka PA, Holmes DR Jr, Edwards WD, Nishimura RA, Tajik AJ, Kyle RA. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med 1988;148:662-6.[Abstract/Free Full Text]
  4. Kyle RA, Spittell PC, Gertz MA, Li CY, Edwards WD, Olson LJ, et al. The premortem recognition of systematic senile amyloidosis with cardiac involvement. Am J Med 1996;171:395-400.
  5. Gertz MA, Kyle RA, Edwards WD. Recognition of congestive heart failure due to senile cardiac amyloidosis. Biomed Pharmacother 1989;43:101-6.[Medline]
  6. Elias JM. Histochemical and immunohistochemical analysis of amyloid. In: Elias JM, editor. Immunohistopathology. A practical approach to diagnosis. Chicago: ASCP [American Society for Clinical Pathology] Press; 1990. p. 441-88.




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