Wild-type (senile) amyloidosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]

Overview

Medical Therapy

  • There is no treatment for wild-type amyloidosis; the mainstay of therapy is supportive treatment aimed at symptoms of the disease.
  • Since wild-type amyloidosis commonly presents with symptoms of right-sided heart failure and carpal tunnel syndrome, treating amyloid deposits in the heart is important.
  • Supportive treatment is with diuretics, antiarrhythmics or pacemaker implantation, anticoagulation where supraventricular arrhythmias are present, and an avoidance of digoxin and calcium channel blockers.
  • Antihypertensives are usually poorly tolerated as these patients can be profoundly hypotensive
  • For some patients with severe wild-type (senile) ATTR amyloidosis, a heart transplant may be the best option.
  • In ATTRwt amyloidosis, therapy is supportive, but both for this disease and for ATTR, pharmacologic therapies aimed at stabilizing the transthyretin molecule and thus preventing amyloid formation are being actively investigated.
  • In patients with transthyretin amyloid cardiomyopathy, Tafamidis was associated with reductions in all-cause mortality and cardiovascular-related hospitalizations and reduced the decline in functional capacity and quality of life as compared with placebo.[1]

References

  1. Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M; et al. (2018). "Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy". N Engl J Med. 379 (11): 1007–1016. doi:10.1056/NEJMoa1805689. PMID 30145929.