Case Session 02: Non-ischemic Cardiomyopathies (2018)-CMR relaxometry in Freidreich ataxia

CMR relaxometry in Freidreich ataxia

Video Transcription

Video Summary

A 25-year-old man with Friedreich ataxia underwent cardiac MRI after dyspnea, hypertension, murmur, ECG LVH, and echocardiographic biventricular hypertrophy. CMR showed asymmetric septal hypertrophy, preserved LV ejection fraction, mild LVOT flow acceleration, mildly elevated septal native T1, and patchy non-ischemic late gadolinium enhancement in the lateral and inferior walls. The case highlights cardiac involvement in Friedreich ataxia, a condition often causing hypertrophic cardiomyopathy and heart failure. Because iron accumulation may be subtle, T1 mapping may detect mild iron-loading when T2* is normal.

Keywords

Friedreich ataxia

hypertrophic cardiomyopathy

cardiac MRI

T1 mapping

late gadolinium enhancement