Abstract #2750

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

Jian-Ling Chen^1,2, Liang-Wei Chen^1,2, Sheng-Che Hung^1,2,3, Hsien-Tzu Liu^1,2, Mei-Han Wu^1,2, Fu-Pang Chang^2,4, An-Hung Yang^2,4, Ting-Rong Hsu^2,5, Dau-Ming Niu^2,5, Ming-Ting Wu^2,6, Chui-Mei Tiu^1,2, and Chien-Yuan Lin^7,8

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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