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Cardiac Amyloidosis (ATTRwt) — EECC MCQ

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HardCardiomyopathyCardiac Amyloidosis (ATTRwt)EECC

A 55-year-old man is diagnosed with wild-type transthyretin cardiac amyloidosis (ATTRwt) following DPD scintigraphy showing Grade 3 uptake, negative serum free light chains, and negative urine Bence-Jones protein. He has NYHA class II symptoms with LVEF 52%. What is the recommended disease-modifying pharmacological therapy?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: C — Tafamidis 80 mg once daily

Explanation lettering: D = shown as B · E = shown as C · B = shown as D · C = shown as E

Per the 2023 ESC Cardiomyopathy Guidelines and NICE TA696, tafamidis (a TTR stabiliser) is recommended for ATTR cardiac amyloidosis in patients with NYHA class I-II, based on the ATTR-ACT trial. Patisiran (B) is a TTR gene silencer for hereditary ATTR with polyneuropathy. Diflunisal (C) is off-label with limited evidence. Chemotherapy (D) is for AL amyloidosis. Doxycycline/UDCA (E) has minimal evidence. Apply this conclusion only to the clinical circumstances stated, with appropriate specialist review, contraindication checks, shared decision-making and follow-up. Reassess if the physiology, treatment response or competing risk changes. The competing options would require a different haemodynamic profile, diagnosis, procedural indication or risk balance from the one described here.

Reference: ESC (2023): Guidelines for the Management of Cardiomyopathies: https://bnf.nice.org.uk/