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法布里病的心脏磁共振特征:从早期诊断到预后分层

Cardiac Magnetic Resonance Features of Fabry Disease: From Early Diagnosis to Prognostic Stratification.

作者信息

Camporeale Antonia, Diano Alberto, Tondi Lara, Pica Silvia, Pasqualin Giulia, Ciabatti Michele, Graziani Francesca, Pieroni Maurizio, Lombardi Massimo

机构信息

Multimodality Cardiac Imaging Section, IRCCS Policlinico San Donato, San Donato Milanese, 20097 Milan, Italy.

Department of Clinical Science and Community Health, University of Milan, 20122 Milan, Italy.

出版信息

Rev Cardiovasc Med. 2022 May 16;23(5):177. doi: 10.31083/j.rcm2305177. eCollection 2022 May.

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

在过去几年中,心脏磁共振成像(CMR)的广泛应用显著改变了法布里病(FD)心脏受累研究的方法。进行无创组织特征分析的可能性,包括诸如T1/T2映射等新序列,为与其他形式的左心室肥厚进行鉴别诊断提供了强大工具。在确诊为FD的患者中,CMR是早期检测心脏受累最敏感的无创技术,它为心脏损伤的演变提供了新的见解,包括性别特异性特征。最后,CMR对心脏形态、功能和组织成分细微变化的多参数检测可能有助于长期监测特定治疗的疗效。本文旨在全面综述目前关于CMR在FD心脏受累中的应用及其临床意义的知识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a82e/11273773/c34a4f8966ae/2153-8174-23-5-177-g1.jpg

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