CMR in Cardiac Amyloidosis — EECC MCQ

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ModerateCardiac ImagingCMR in Cardiac AmyloidosisEECC

A 70-year-old man with unexplained LVH undergoes cardiac MRI. Native T1 values are markedly elevated (1200 ms at 1.5T, normal ~950-1050), ECV is 65% (normal <30%), and there is diffuse, circumferential LGE. What diagnosis do these findings most strongly suggest?

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Correct answer: D — Cardiac amyloidosis — very high T1 and ECV values with diffuse LGE are characteristic; ECV >40% is highly suggestive, and values >55% are almost pathognomonic for amyloidosis

Cardiac amyloidosis has a distinctive CMR signature: (1) markedly elevated native T1 (the highest among all cardiomyopathies); (2) dramatically elevated ECV (typically >40%, often >55% — reflecting massive extracellular expansion from amyloid deposition); (3) characteristic LGE pattern — diffuse, subendocardial or transmural, often circumferential, with difficulty nulling the myocardium (abnormal gadolinium kinetics). The 2023 ESC Cardiomyopathy Guidelines recognise CMR T1 mapping and ECV as powerful non-invasive markers for amyloidosis. ECV in particular correlates with amyloid burden and prognosis. Iron overload shows the opposite pattern (very LOW T1 and T2*). Hypertensive LVH has modestly elevated T1/ECV. These parametric mapping differences enable non-invasive tissue differentiation.

Reference: ESC (2023): Cardiomyopathies Guidelines; SCMR Mapping Consensus