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.
Key red flags
Rapid HF progression, hypotension and disproportionate troponin/NT-proBNP with any monoclonal abnormality requires urgent specialist assessment.
Investigation priorities
Screen sensitively for a monoclonal protein and possible AL.
Management branches
Cardiac imaging plus clinical red flags.
- Obtain ECG/echo and send serum free light chains, serum immunofixation and urine immunofixation together; arrange CMR where it will refine phenotype.
- 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.
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.
Acoramidis712 mg orally twice daily (two 356 mg tablets twice daily).