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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Infiltrative cardiomyopathy and cardiac amyloidosis

Cardiology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
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Escalate

Suspected AL amyloidosis with cardiac involvement is time-critical: urgent haematology/amyloidosis-centre assessment is required. Syncope, high-grade block, shock or rapidly worsening congestion also needs urgent admission.

Synopsis

Recognise infiltrative red flags, separate AL from ATTR safely and start subtype-specific treatment without avoidable delay.

  • Think amyloid when increased wall thickness is paired with low-voltage discordance, restrictive physiology, apical-sparing strain or systemic red flags.
  • ATTR red flags include bilateral carpal tunnel, lumbar spinal stenosis, biceps rupture, neuropathy and unexplained HFpEF or aortic stenosis in later life.
  • First exclude a monoclonal process with serum free light chains plus serum and urine immunofixation; electrophoresis alone is insufficient.
  • Grade 2-3 DPD cardiac uptake can establish ATTR non-invasively only when AL has been excluded and SPECT confirms myocardial uptake.
  • A monoclonal protein does not prove AL and a positive DPD scan does not override it: obtain urgent haematology input and tissue typing.
  • After ATTR diagnosis, sequence TTR even in an older patient because hereditary disease changes family counselling.
  • Diuretics are central for congestion, but hypotension, autonomic dysfunction and a small stiff ventricle often limit conventional HF drugs.
  • NICE-funded ATTR-CM stabilisers now include tafamidis and acoramidis; confirm subtype and eligibility before prescribing.

Key red flags

Time-critical AL

Rapid HF progression, hypotension and disproportionate troponin/NT-proBNP with any monoclonal abnormality requires urgent specialist assessment.

Electrical instability

Syncope, symptomatic bradycardia, high-grade block or ventricular arrhythmia needs monitored assessment; sudden death may be electromechanical as well as tachyarrhythmic.

Investigation priorities

01
Serum free light-chain ratio plus serum and urine immunofixationFirst step

Screen sensitively for a monoclonal protein and possible AL.

Management branches

SuspectedAmyloid diagnostic fork

Cardiac imaging plus clinical red flags.

  1. Obtain ECG/echo and send serum free light chains, serum immunofixation and urine immunofixation together; arrange CMR where it will refine phenotype.
  2. If any monoclonal test is abnormal, refer urgently to haematology/amyloidosis specialists for clonal evaluation and biopsy typing; do not declare ATTR from DPD alone.
  3. If all monoclonal tests are negative, perform DPD scintigraphy with SPECT; grade 2-3 myocardial uptake can support non-biopsy ATTR-CM.
  4. If DPD is grade 0-1, imaging is discordant or the clinical suspicion remains high, pursue expert biopsy/alternative infiltrative diagnoses rather than closing the case.

Key medicines

Tafamidis61 mg orally once daily (tafamidis free acid); this corresponds to 80 mg tafamidis meglumine and the formulations are not interchangeable mg-for-mg.Confirm ATTR and exclude AL first; specialist initiation. Limited evidence in very advanced NYHA IV disease; reassess around transplantation and pregnancy.
Acoramidis712 mg orally twice daily (two 356 mg tablets twice daily).Specialist initiation after subtype confirmation; limited data if CrCl below 30 mL/min and none on dialysis, so use caution. Not recommended in hepatic impairment; an early eGFR change may reflect haemodynamics.
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Sources and review status6 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • ESC: Cardiac amyloidosis—epidemiology, diagnosis and therapyMonoclonal testing, scintigraphy and biopsy algorithm.
  • Leeds Teaching Hospitals: Cardiac Amyloidosis ServiceCurrent February 2026 NHS referral tests and AL exclusion.
  • NICE TA984: Tafamidis for ATTR-CMNHS eligibility for tafamidis.
  • Vyndaqel 61 mg SmPCExact tafamidis dose and diagnostic prerequisite.
  • NICE TA1121: Acoramidis for ATTR-CMPublished January 2026; NHS eligibility for acoramidis.
  • Beyonttra 356 mg SmPCExact acoramidis dose and renal/hepatic cautions.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom