Cardiac amyloidosis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3]; Cafer Zorkun, M.D., Ph.D. [4]; Lakshmi Gopalakrishnan, M.B.B.S. [5]


The presence or absence of cardiac involvement with amyloid is the most important prognostic factor.[1] Untreated cardiac amyloidosis is associated with a very poor prognosis and a high mortality rate. The most common cardiac complications include heart failure, sudden cardiac death due to electromechanical dissociation and pericardial effusion.

Natural History

Untreated cardiac amyloidosis is associated with high rate of cardiac complications and eventually death. Cardiac involvement in AL amyloidosis is associated with a higher mortality rate compared with that in senile amyloidosis and familial forms of amyloidosis. Also, heart failure in AL type amyloidosis of the heart is more difficult to treat as compared with that associated with TTR type amyloid.


The following are the complications of cardiac amyloidosis:


Cardiac amyloidosis is a chronic and progressive condition. A cardiologist may estimate the prognosis according to the thickness of the left ventricle and to the degree of restriction in the heart (diastolic dysfunction). Cardiac involvement is the most important predictor of survival in patients with AL amyloidosis.

Mayo Staging System

Studies have shown that cardiac torponin T and N-terminal proBNP are powerful prognostic indicators in AL amyloidosis. Based on the combined levels of both the indicators a staging system has been developed for risk stratification of patients with AL amyloidosis. Two prognostic models for risk stratification were designed using threshold values for torponins and NT-proBNP. The threshold values used for the markers were: NT-proBNP <332 ng/L, cTnT <0.035 μ<g/L, and cTnI <0.1 <μg/L.[14][15]

Stage NT-proBNP cTnT/cTnI Median Survival (months)
Stage I Low Low 26.4 - 27.2
Stage II Low or Elevated Elevated or Low 10.5 - 11.1
Stage III Elevated Elevated 3.5 - 4.1

In 2009 another staging system was proposed incorporating serum free light chain levels into the Mayo staging system. The threshold values used were: NT-proBNP <332 ng/L, cTnT <0.035 μ<g/L, and serum FLC <500mg/L. As the stage increased it was observed that survival rates reduced drastically.

Stage NT-proBNP cTnT Serum FLC
Stage I Normal Normal Low
Stage II High Normal Low
Stage III High Normal High
Stage IV High High High

The presence of nodular deposits, thick perimyocytic layers of amyloid, and small myocyte diameters in an endomyocardial biopsy are associated with the worst prognosis. [16]


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