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ATTRwt vs ATTRv Differences — EECC MCQ

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ModerateCardiomyopathyATTRwt vs ATTRv DifferencesEECC

A 55-year-old man with wild-type ATTR cardiac amyloidosis (ATTRwt) has been started on tafamidis 61 mg (tafamidis free acid). His symptoms stabilise. How does ATTRwt differ from hereditary ATTR (ATTRv) in terms of demographics and presentation?

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Correct answer: C — ATTRwt predominantly affects men over 65, presents with HFpEF phenotype with LVH and carpal tunnel syndrome, and has no identifiable TTR mutation — it is caused by age-related wild-type TTR misfolding

ATTRwt (previously called 'senile cardiac amyloidosis') results from age-related misfolding and deposition of normal (wild-type) transthyretin protein. Key differences from hereditary ATTR (ATTRv): (1) Demographics: ATTRwt predominantly affects men >65-70 years (male:female ~20:1 vs ~1:1 in ATTRv); (2) Genetics: no TTR mutation (genetic testing negative); (3) Phenotype: primarily cardiac (HFpEF with LVH, diastolic dysfunction), often preceded by bilateral carpal tunnel syndrome and lumbar spinal stenosis; polyneuropathy is rare (unlike ATTRv where neuropathy is common); (4) Prevalence: increasingly recognised as a common cause of HFpEF in the elderly (found in 13% of HFpEF hospitalisations and 16% of patients undergoing TAVI). Tafamidis is effective in both ATTRwt and ATTRv cardiac amyloidosis.

Reference: ESC (2023): Cardiomyopathies Guidelines