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Cardiac Amyloidosis (ATTR) — EECC MCQ

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HardCardiomyopathyCardiac Amyloidosis (ATTR)EECC

A 75-year-old man with progressive heart failure (LVEF 45%), bilateral carpal tunnel syndrome, and lumbar spinal stenosis has an ECG showing low voltages with a pseudo-infarct pattern. Echocardiography reveals concentric LV hypertrophy (wall thickness 16 mm) with granular sparkling and grade II diastolic dysfunction. What is the gold-standard non-invasive investigation to confirm the suspected diagnosis?

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Correct answer: A99mTc-DPD bone scintigraphy

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

The clinical features strongly suggest transthyretin cardiac amyloidosis (ATTR-CA). Per the 2023 ESC Cardiomyopathy Guidelines, 99mTc-DPD bone-tracer scintigraphy is the gold-standard non-invasive diagnostic test for ATTR-CA (Grade 2-3 uptake has above 99% specificity when serum free light chains and monoclonal protein are negative). CMR (A) shows characteristic patterns but cannot definitively distinguish ATTR from AL. Serum FLC (D) excludes AL but does not confirm ATTR. Genetic testing (E) distinguishes hereditary from wild-type ATTR but comes after diagnosis. PET-CT (C) is for sarcoidosis.

Reference: ESC (2023): Guidelines for the Management of Cardiomyopathies