Multiple Choice Question of the Week : CMR-01 : [Answer]

             
      



61 years old woman, episodes of chest pain, absence of coronary artery disease at ICA. No other diseases. No hypertension. No familial diseases Known. EKG: ST abnormalities in V4-V6.

Which is the best interpretation of these images?
A – Normal for age
B – Fabry disease
C – Myocarditis
D – Amyloidosis

Right answer is B: Fabry disease

Mild hypertrophy in no hypertension patient, LGE pattern with diffuse areas of intramyocardial gadolinium retention and, especially, reduced T1 values (850 ms) in T mapping sequences are indicative of intracellular storage disease. Fabry disease was confirmed by genetic analysis.

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