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Abstract #4710

Cardiovascular magnetic resonance findings in Danon disease:A case series of a big family

Xiaolong Liu1, Zhanguo Sun1, Ning Zhai1, and Xiuzheng Yue2
1Department of Radiology, Affiliated Hospital of Jining Medical University, Jining, China, 2Philips Healthcare (Beijing), Beijing, China

Synopsis

Keywords: Heart, MyocardiumHeart involvement is the leading cause of death of Danon disease (DD). However, the cardiac magnetic resonance (CMR) features of DD are not well understood. Hence, we present seven DD patients with from the same family with follow-up since 2017. In our cohort, the typically CMR features of DD were left ventricle (LV) hypertrophy, late gadolinium enhancement of LV, diminished strain, and preserved or reduced LV ejection fraction. Late gadolinium enhancement most commonly involves the LV free wall and the right ventricle insertion points. Moreover, these CMR changes in male patients appeared earlier and more significant, and progressed faster.

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Keywords