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Cardiac Amyloidosis - CMR Findings — EECC MCQ

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HardCardiac ImagingCardiac Amyloidosis - CMR FindingsEECC

A 52-year-old woman with suspected cardiac amyloidosis undergoes cardiac MRI. Native T1 mapping shows markedly elevated values (1180 ms at 1.5T, normal below 1060 ms). T2 mapping is normal. Extracellular volume (ECV) fraction is 55% (normal below 30%). LGE shows diffuse subendocardial enhancement with difficulty nulling the myocardium. What do these findings indicate?

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Correct answer: A — Cardiac amyloidosis with significant amyloid burden

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

Markedly elevated native T1, markedly elevated ECV fraction (reflecting expanded extracellular space from amyloid deposition), diffuse subendocardial LGE with difficulty nulling, and normal T2 are pathognomonic for cardiac amyloidosis. Myocarditis (A) would show elevated T2. Fabry disease (C) shows low T1 values. Iron overload (D) shows low T2* and T1 values. Hypertensive heart disease (E) would show mild T1 elevation and normal ECV.

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