This handout is for adhf in cardiac amyloidosis (attr-cm and al). Your care team identified this based on: hf symptoms + lv wall thickness ≥12 mm + no long-standing htn → amyloidosis screen.
Other reasons your team may use this plan: hf + low-voltage qrs on ecg + thick lv walls (voltage-mass mismatch — amyloid signature); elderly male with bilateral carpal tunnel release history + new hf (attrwt clue); monoclonal protein on spep/sflc + hf symptoms → al amyloidosis emergent workup.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| tafamidis | 61 mg PO daily (free acid; equivalent to tafamidis meglumine 80 mg) | PO | daily lifelong | ATTR-ACT (Maurer NEJM 2018; PMID 30145930) — 30% all-cause mortality reduction over 30 mo; greatest benefit NYHA I–II; less impact NYHA III |
| patisiran | 0.3 mg/kg IV every 3 weeks (premedicate with dex/H1/H2/acetaminophen) | IV | every 3 weeks | APOLLO (Adams NEJM 2018; PMID 30144829) — siRNA reduces hepatic TTR production |
| vutrisiran | 25 mg SC every 3 months | SC | every 3 months | HELIOS-A (Adams Lancet Neurol 2023; PMID 35262029) — SC dosing convenience |
| daratumumab | 1800 mg SC weekly × 8, then biweekly × 16, then monthly (with dex + bortezomib + cyclophosphamide — D-VCd regimen) | SC | per ANDROMEDA schedule | ANDROMEDA (Kastritis NEJM 2021; PMID 34077641) — daratumumab + CyBorD improved hematologic CR + organ response in AL |
| bortezomib | 1.3 mg/m² SC weekly (with cyclophosphamide + dexamethasone — CyBorD) | SC | weekly cycles per oncology | CyBorD backbone for AL (proteasome inhibition kills plasma cells); base regimen prior to ANDROMEDA daratumumab era |
| furosemide | 20–40 mg IV/PO (gentle — narrow preload window in restrictive physiology) | IV/PO | daily | Restrictive amyloid is preload-dependent; over-diuresis precipitates hypotension/syncope; titrate carefully |
| midodrine | 5–10 mg PO TID (last dose before 6 PM to avoid supine HTN) | PO | TID | Amyloid autonomic neuropathy → orthostasis; midodrine increases vascular tone without bradycardia |
Plan: Cardiac amyloidosis ADHF — AL vs ATTR phenotype-based regimen (ESC 2023 PMID 37596926)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Guideline: ESC 2023 cardiac amyloidosis position statement + ACC/AHA 2023 expert decision pathway (Kittleson) + ATTR-ACT + ANDROMEDA + APOLLO + HELIOS-A