Cardiac amyloidosis is managed by addressing both the cardiac manifestations and the underlying disease.
Management of the cardiac manifestations is aimed at relieving the symptoms of heart failure. Cautious diuresis is used to relieve congestion while preserving preload.1,10 Treatment of arrhythmias is vital but also problematic. Atrial kick is necessary to maintain left ventricular preload.9
-Blockers and calcium channel blockers are contraindicated because of their negative inotropic effect.1,10 Patients with cardiac amyloidosis should not take digoxin because of the risk of toxic effects related to binding of the drug to amyloid fibrils.
1,10 The use of pacemakers and/or implantable cardioverterdefibrillators is controversial, as these interventions are known to improve symptoms but not survival in patients with cardiac amyloidosis.
1 Anticoagulation is indicated in the presence of atrial fibrillation because of an increase in thromboem embolism events.10 Pleural effusions may require thoracentesis and/or a chest tube but often prove to be resistant to therapy.10 AL amyloidosis is treated with antiplasma cell therapy. The most common treatment for AL amyloidosis is the combination of melphalan (a chemotherapeutic agent) and prednisone.5Melphalan is an antineoplastic medication that is classified as a nitrogen mustard. Nitrogen mustards result in a reaction with deoxyribonucleic acid (DNA) molecules called alkylation. Alkylated DNA cannot replicate properly and cell death results. Autologous stemcell transplantation with high-dose