Cardiac Amyloidosis Treatment & Management

Updated: May 09, 2019
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Terrence X O'Brien, MD, MS, FACC  more...
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Treatment

Approach Considerations

Management of amyloidosis includes supportive measures for symptomatic relief, management of congestive heart failure, and specific measures toward underlying amyloid disease. Patients with large pleural effusions may need thoracentesis.

The US Food and Drug Administration (FDA) approved tafamidis meglumine (Vyndaqel) and tafamidis (Vyndamax) for cardiomyopathy caused by transthyretin-mediated amyloidosis (ATTR-CM) in adults in May 2019. [72] These are the first FDA-approved treatments for ATTR-CM. Although each product has the same active moiety, tafamidis, they are not substitutable on a milligram-to milligram basis and their recommended doses differ. [72]

The efficacy of tafamidis was shown in a clinical trial of 441 patients randomized to receive tafamidis meglumine or a placebo. [72, 73] All-cause mortality and rates of cardiovascular-related hospitalizations were lower among the 264 patients who received tafamidis than among the 177 patients who received placebo (P< 0.001). [73] After an average of 30 months, tafamidis was also associated with a lower rate of decline in distance for the 6-minute walk test (< 0.001) and a lower rate of decline in Kansas City Cardiomyopathy Questionnaire–Overall Summary (KCCQ-OS) score (P< 0.001). [73]

Hospitalization may be needed for management of heart failure. Selected patients may require transfer to the centers where facilities for chemotherapy are available.

Patients should be advised to take a salt-restricted diet as well as check their weights on a regular basis to assess for volume status. Activity can be performed as tolerated

Waist-high elastic stockings may be useful in patients with orthostatic hypotension.

Follow-up is performed in the heart failure clinic. No preventive strategies are applicable to primary amyloidosis. Secondary amyloidosis improves after treatment of underlying condition.

Consultations

Surgical consultation for abdominal fat pad or rectal biopsy is suggested. Other suggested consultations include the following:

  • Hematology/oncology specialists

  • Cardiology consultation for management of heart failure

  • Electrophysiology consultation for pacemaker implantation when clinically indicated