Hypertrophic cardiomyopathy with fibrosis

Case contributed by Fabien Ho

Presentation

Known familial history of hypertrophic cardiomyopathy.

Patient Data

Age: 30 years
Gender: Male

Cardiac MRI

mri

LVEF 44%. Myocardial mass 162g/m², very high.

Sarcomeric hypertrophic cardiomyopathy, asymmetric form, predominantly on the septum: thickened 30mm septum with patchy LGE on septum and anteroseptal wall consistent with fibrosis. Lateral wall is also thickened up to 20mm, as well as the RV apical wall up to 15mm.

No SAM, no subaortic obstruction, no midventricular obstruction.

Case Discussion

Such a severe form at an early age suggests an early onset of the disease. 

Familial history, patient's young age, T1 mapping, and Ti value (not shown) ruled out amyloidosis or other infiltrative cardiomyopathies.

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