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IMAGES Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 842-843
Cardiac amyloidosis: Report of an autopsy case with review of the literature


1 Department of Pathology, M. S. Ramaiah Medical College, Bangalore - 560 054, Karnataka, India
2 Department of Forensic Medicine, M. S. Ramaiah Medical College, Bangalore - 560 054, Karnataka, India

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Date of Web Publication27-Oct-2010
 

How to cite this article:
Mysorekar VV, Rao SG, Satish N T, Kamath SM. Cardiac amyloidosis: Report of an autopsy case with review of the literature. Indian J Pathol Microbiol 2010;53:842-3

How to cite this URL:
Mysorekar VV, Rao SG, Satish N T, Kamath SM. Cardiac amyloidosis: Report of an autopsy case with review of the literature. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Dec 6];53:842-3. Available from: http://www.ijpmonline.org/text.asp?2010/53/4/842/72077


Amyloidosis comprises a group of diseases that share in common, the extracellular deposition of insoluble fibrillar proteins in organs and tissues. [1] The spectrum of organ involvement can include the kidneys, heart, blood vessels, central and peripheral nervous systems, liver, intestines, lungs, eyes, skin, and bones. [2] When amyloidosis affects the heart, a devastating and progressive process can lead to restrictive cardiomyopathy, congestive cardiac failure, arrhythmias, conduction abnormalities, angina and death. [3] We describe one such case.

A medicolegal autopsy was done on a 42-year-old female who had suddenly collapsed and was brought dead to the hospital. Her relatives reported a history of weakness, fatigue, dyspnea, chest pain and bilateral pedal edema since 1 year, for which she had been taking symptomatic treatment.

Both the lungs were heavy and sectioning revealed frothy, blood-tinged fluid. The heart was enlarged and weighed 550 g. It was firm in consistency. The right and left ventricular walls were 1.0 and 1.8 cm in thickness, respectively, and the inter-ventricular septum had a thickness of 1.8 cm [Figure 1]a. The chambers, valves and great vessels were normal. The coronary arteries were patent. The other organs were grossly unremarkable.
Figure 1: (a) Cross section of the heart showing the thickened interventricular septum and left ventricular wall. (b) Photomicrograph of the left ventricular myocardium showing large homogeneous, eosinophilic deposits separating the muscle fibers (H and E, ×400)

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The lungs showed congestion of the alveolar septal capillaries, and intra-alveolar eosinophilic fluid, suggesting pulmonary edema. The myocardium showed homogeneous, eosinophilic deposits separating the muscle fibers and in the walls of the blood vessels [Figure 1]b. The liver, spleen and kidney showed eosinophilic deposits in the walls of the blood vessels [Figure 2]a,b. Congo red staining followed by polarizing microscopy demonstrated an apple-green birefringence of these deposits, confirming that they were amyloid deposits. The other organs were microscopically normal. A final diagnosis of systemic amyloidosis with signs of congestive cardiac failure was made. The immediate cause of death was most likely to have been an acute myocardial ischemic episode resulting from microvasculature disease, or cardiac arrhythmia due to involvement of the conduction system.
Figure 2: (a) Photomicrograph of the liver showing pink red deposits in the walls of the blood vessels (Congo red, ×400). (b) Photomicrograph of the kidney showing homogeneous, eosinophilic deposits in the walls of the blood vessels (H and E, ×400)

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Cardiac symptoms are often the first indicator of systemic amyloidosis. [4] Five different types of amyloidosis have been described according to the underlying disease: [2],[5]

  1. Primary amyloidosis (AL amyloid) where there is a plasma cell disorder,
  2. Reactive systemic or secondary amyloidosis (AA amyloid) which is associated with a chronic infection or inflammation; cardiac involvement is not severe in these cases,
  3. Familial amyloidosis associated with transthyretin gene mutations; a family history of amyloidosis is present in these cases,
  4. Senile systemic amyloidosis which affects the heart in 25% patients older than 80 years. This type of amyloidosis mainly involves the atria (91%), and
  5. Hemodialysis-associated amyloidosis in patients with chronic renal failure who are on hemodialysis.


In our case, as no underlying chronic infection was detected at autopsy, secondary amyloidosis was ruled out. On account of the absence of a family history of amyloidosis, familial amyloidosis was not likely. Our patient was not old enough to be suffering from senile amyloidosis and did not have severe atrial involvement as in these cases. She had not been on hemodialysis. Thus, having ruled out all the other types of amyloidosis, we were left with the possibility of primary amyloidosis, although there was no obvious evidence of a plasma cell disorder, at autopsy. The great majority of patients with AL amyloid do not have classic multiple myeloma or any other overt B-cell neoplasm. [1] In virtually all such cases, however, monoclonal immunoglobulins or free light chains, or both, can be found by urine or serum protein immunofixation electrophoresis. [1] Unfortunately, our patient had not been investigated in detail. We also do not know whether she had biochemical signs of renal or liver dysfunction; however, this seems unlikely as the amyloid deposits in the kidney and liver involved only the blood vessels, without much of parenchymal replacement.

Methods for diagnosis of amyloidosis include abdominal fat aspiration, rectal or gingival biopsy, endomyocardial biopsy, electrocardiography (ECG) and echocardiography. [2],[4],[5]

The prognosis of primary amyloidosis is poor. The median survival from the time of onset of congestive cardiac failure is only 6 months.

In these days of the "dying autopsy", it is unusual to get an opportunity to do a detailed study of all the organs in a case of systemic amyloidosis. The present case emphasizes the usefulness of an autopsy in determining a definite cause of death, which would otherwise have gone undetected.


   Acknowledgment Top


The authors are grateful to Dr. S. Kumar, Principal, M. S. Ramaiah Medical College, for his kind support for publishing this paper.

 
   References Top

1.Abbas AK. Diseases of immunity. In: Kumar V, Abbas AK, Fausto N, editors. Robbins and Cotran Pathologic basis of disease. 7 th ed. Philadelphia: Saunders; 2004. p. 193-267.  Back to cited text no. 1
    
2.Shah KB, Inoue Y, Mehra MR. Amyloidosis and the heart: A comprehensive review. Arch Intern Med 2006;166:1805-13.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Eshaghian S, Kaul S, Shah PK. Cardiac amyloidosis: new insights into diagnosis and management. Rev Cardiovasc Med 2007;8:189-99.  Back to cited text no. 3
[PUBMED]    
4.Maredia N, Ray SG. Cardiac amyloidosis. Clin Med 2005;5:504-9.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Hassan W, Al-Sergani H, Mourad W, Tabbaa R. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis and management. Tex Heart Inst J 2005;32:178-84.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Vijaya V Mysorekar
89, A.G.'s office colony, 5th Main, 6th Cross, New BEL Road, Bangalore - 560 054, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72077

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