Evolution and prognostic significance of heart involvement in patients with systemic AL-amyloidosis

Aim

To study the prognostic significance of heart involvement in patients with systemic AL-amyloidosis.

Material and methods

We conducted a retrospective study in consecutive patients with systemic AL-amyloidosis, which were admitted to our clinic in 1995-2017. Diagnosis of amyloidosis was verified histologically by Congo red staining and polarized microscopy.

Results

In patients with AL-amyloidosis, chronic heart failure and orthostatic hypotension were associated with the worst survival and the highest risk of death. In the later term, a higher mortality coincided with development of chronic renal failure that reduced a prognostic significance of chronic heart failure. At this time, expected survival in patients with and without orthostatic hypotension and/or chronic renal failure was 28 vs. 123 months, respectively (р=0.00007). Multi fac torial analysis showed that the use of bortezomib-based rapidly acting regimens of treatment is the only possible approach to improve prognosis in patients with AL-amyloidosis.

Conclusion

Orthostatic hypotension and chronic heart failure are associated with poor prognosis in patients with AL-amyloidosis. However, their unfavorable effect is mediated by chronic renal failure.

Key words

AL, amyloidosis, monoclonal gammopathy, plasmatic dyscrasia, cardiomyopathy, chronic renal failure, chronic renal disease, orthostatic hypotension, bortezomib.