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Abstract：
In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication.
摘要：
在过去的几年里,心脏磁共振（CMR）的广泛应用极大地改变了法布里病（FD）心脏受累的研究方法。进行非侵入性组织表征的可能性，包括新的序列，如T1/T2映射，为与其他形式的左心室肥厚的鉴别诊断提供了有力的工具。在确诊为FD的患者中，CMR是早期检测心脏受累最敏感的非侵入性技术，它为心脏损伤的演变提供了新的见解，包括性别特征。最后,CMR多参数检测心脏形态的细微变化，随着时间的推移，功能和组织成分可能有助于监测特定治疗的疗效。本文旨在对有关CMR在FD心脏受累中的应用及其临床意义的最新知识进行全面综述。
