Sensitivity of STEAM DT-CMR diastolic secondary eigenvector angle to abnormal microarchitecture in early-stage hypertrophic cardiomyopathy

Motivation: Improve assessment of early-stage hypertrophic cardiomyopathy (HCM) by MRI. Goal(s): That DT-CMR can identify cardiac microstructural abnormalities in young variant carriers before the onset of symptoms or hypertrophy in HCM, and that these changes may not occur in their genetically unaffected siblings. Approach: Early stage sarcomere gene variant carriers were recruited; 30 SARC+ and 30 SARC- controls. Cardiac diffusion tensor imaging was performed to assess sensitivity of diffusion to early-stage HCM. Results: Among the DT-CMR parameters in SARC+ and SARC-control subjects, the diastolic E2A mean over the SA slice was significantly higher in the SARC+ cohort compared to the SARC- group. Impact: HCM is linked to the risk of sudden cardiac death and heart failure. There are now novel treatments that may cure HCM. Our study highlights the role of DT-CMR in identifying individuals who may benefit from these treatments.