Patient handout

Cardiogenic shock — cardiac amyloidosis (ATTR-CM and AL)

PRODUCTION

1. Your condition

This handout is for cardiogenic shock — cardiac amyloidosis (attr-cm and al). Your care team identified this based on: cardiogenic shock + lv wall thickness ≥12 mm + no long-standing htn → cardiac amyloidosis with shock physiology.

Other reasons your team may use this plan: cardiogenic shock + low-voltage qrs on ecg + thick lv walls (voltage-mass mismatch — pathognomonic amyloid signature); known cardiac amyloidosis (attr or al) decompensating from chronic hf to shock physiology (sbp <90, lactate ≥2); monoclonal protein on spep/sflc + shock physiology → al amyloidosis emergent oncologic workup.

2. Your medications

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
norepinephrine	0.05–0.5 µg/kg/min titrate MAP ≥65	IV	continuous	SOAP-II PMID 20200382 — NE first-line vasopressor in CS; preferred over dobutamine in amyloid (typical inotropes paradoxically worse — worsen LVOT obstruction, ventricular arrhythmia, autonomic instability)
phenylephrine	0.5–2 µg/kg/min titrate	IV	continuous	Pure-alpha vasopressor — useful in amyloid CS when beta-stimulation precipitates arrhythmia or worsens LVOT gradient; avoids tachyarrhythmia exacerbation
tafamidis	61 mg PO daily lifelong	PO	daily	ATTR-ACT (Maurer NEJM 2018 PMID 30145930) — 30% mortality reduction; benefit takes 12-30 months → MCS bridge needed for shock presentation; recommended for ALL ATTR-CM with NYHA I-III
patisiran	0.3 mg/kg IV q3 weeks (max 30 mg)	IV	q3 weeks	APOLLO PMID 30144829 — siRNA TTR silencer for ATTRv polyneuropathy; trial-evidence base extending to cardiac endpoints (APOLLO-B PMID 36867222)
vutrisiran	25 mg SC q3 months	SC	q3 months	HELIOS-A (PMID 35262029) — SC vutrisiran q3 mo, more convenient than patisiran IV; HELIOS-B cardiac endpoint trial supportive
daratumumab	1800 mg SC weekly cycles 1-2, q2w cycles 3-6, q4w after	SC	weekly to monthly	ANDROMEDA PMID 34077641 — Dara-CyBorD vs CyBorD alone significantly improved hematologic complete response in newly diagnosed AL; first-line for AL amyloidosis per ISA 2024 consensus
bortezomib	1.3 mg/m2 SC weekly cycles 1-2, then per protocol	SC	weekly	CyBorD backbone for AL amyloidosis; proteasome inhibitor for plasma-cell dyscrasia; SC route preferred over IV for neuropathy reduction
cyclophosphamide	300 mg/m2 PO weekly per CyBorD protocol	PO	weekly	CyBorD backbone for AL amyloidosis; alkylating cytotoxic for plasma-cell dyscrasia
dexamethasone	40 mg PO weekly per CyBorD protocol (20 mg if frail)	PO	weekly	CyBorD backbone steroid; dose-reduce in elderly / frail / cardiac stage IV
furosemide	GENTLE 20–40 mg IV titrate slowly	IV	bolus or low-dose continuous	CAUTION — preload-dependent restrictive physiology; gentle diuresis only if overt congestion + adequate perfusion; large-volume diuresis precipitates hypotension and AKI (ESC 2023 cardiac amyloidosis statement PMID 37596926)
midodrine	5 mg PO TID titrate to 10 mg TID	PO	TID	Oral alpha-agonist for amyloid orthostasis (autonomic dysfunction); bridge during pressor wean post-CS recovery

Plan: Amyloid CS — phenotype-directed disease-modifying therapy + CS support; MCS bridge to therapy onset (months) or transplant

3. When to call your provider

Contact your care team if any of the following happen:

Recurrent shock → ED + CICU + MCS evaluation
AL relapse on FLC → hematology + second-line therapy
ATTR progression → add gene silencer + transplant evaluation
New high-grade AV block → EP urgent (rare in amyloid but reported)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Patient with cardiac amyloidosis inadvertently prescribed digoxin (binds amyloid fibrils → toxicity at therapeutic-range serum levels) — emergency: stop digoxin, supportive care, digibind for severe toxicity(life-threatening)
Refractory amyloid CS despite NE + IABP — escalate to Impella 5.5 or VA-ECMO bridge to disease-modifying therapy onset (months) or transplant; LV unable to recover quickly without therapy onset(life-threatening)
NYHA IV amyloid CS bridged on MCS — transplant decision window 7-14 days; combined heart-liver transplant evaluation if ATTRv (liver produces 95% of TTR)
AL amyloidosis with cardiac stage III-IV (natural marker of fluid overload (NT-proBNP) >1800, troponin >0.025, dFLC >18) at presentation — oncologic emergency with median survival <6 months without urgent Dara-CyBorD; treatment must start within 48-72 h(life-threatening)
Ventricular arrhythmia storm in amyloid CS (≥3 sustained VT episodes in 24 h) — high mortality; AVOID typical AAD given QT prolongation in amyloid; consider amiodarone with QT monitoring + beta-stimulation withdrawal + EP consult(life-threatening)

5. Follow-up

Cardiology amyloid center quarterly; advanced HF / transplant team monthly if MCS-dependent; hematology q2-4w during AL chemo; ID surveillance during AL therapy (PJP prophylaxis); family ATTRv screening; cardiac rehab if MCS recovery achieved

6. Sources

Guideline: ESC 2023 cardiac amyloidosis position statement (PMID 37596926); ATTR-ACT (Maurer NEJM 2018, PMID 30145930); ANDROMEDA (Kumar PMID 34077641); SCAI 2022 CS staging (Naidu PMID 35718438); Kittleson ACC/AHA 2023 cardiac amyloidosis expert decision pathway