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Abstract #2750

Cardiomyopathy in later-onset Fabry disease: a correlative study of T1 mapping on MR and histology

Jian-Ling Chen1,2, Liang-Wei Chen1,2, Sheng-Che Hung1,2,3, Hsien-Tzu Liu1,2, Mei-Han Wu1,2, Fu-Pang Chang2,4, An-Hung Yang2,4, Ting-Rong Hsu2,5, Dau-Ming Niu2,5, Ming-Ting Wu2,6, Chui-Mei Tiu1,2, and Chien-Yuan Lin7,8

1Radiology, Taipei Veterans General Hospital, Taipei, Taiwan, 2School of Medicine, National Yang Ming University, Taipei, Taiwan, 3Department of Medical Imaging and Radiological Sciences, National Yang Ming University, Taipei, Taiwan, 4Pathology, Taipei Veterans General Hospital, Taipei, Taiwan, 5Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan, 6Radiology, Kaohsiung Veterans Genertal Hospital, Kaohsiung, Taiwan, 7GE Healthcare, Taipei, Taiwan, 8GE Healthcare MR Research China, Beijing, China

Fabry disease is a rare and X-linked disorder characterized by accumulation of glycosphingolipid within lysosomes and resultant multiple organ damage including heart. Since lipid is known to shorten the MRI parameter T1, non-contrast T1 mapping has emerged as key imaging modality to assess Fabry cardiomyopathy and early detection of lipid deposition. This study provides a histologic validation of 7 male patients of untreated later-onset Fabry disease to demonstrate the negative correlation between native myocardial T1 value and severity of lipid deposition (correlation coefficient, -0.771; p, 0.042) and justifies the application of T1 mapping as a noninvasive predictor of surveillance strategy.

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