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用于法布里心肌病临床前评估的多模态成像

Multimodality imaging for pre-clinical assessment of Fabry's cardiomyopathy.

作者信息

Madonna Rosalinda, Cevik Cihan, Cocco Nino

机构信息

Texas Heart Institute and St Luke's Episcopal Hospital, Houston, TX, USA Institute of Cardiology, 'G. D'Annunzio' University, Chieti, C/o Ospedale SS. Annunziata, Via dei Vestini, 66013 Chieti, Italy

Texas Heart Institute and St Luke's Episcopal Hospital, Houston, TX, USA.

出版信息

Eur Heart J Cardiovasc Imaging. 2014 Oct;15(10):1094-100. doi: 10.1093/ehjci/jeu080. Epub 2014 Jun 5.

Abstract

Anderson Fabry's disease (AFD) is a rare but underdiagnosed intracellular lipid disorder which can cause left ventricular hypertrophy (LVH). Pre-clinical diagnosis of Fabry's disease is important as it permits early stratification for enzyme replacement therapy, improving the patient's long-term prognosis, avoiding progression to irreversible fibrosis, and preventing cardiovascular complications. Combinations of imaging modalities that integrate the strengths of each modality and at the same time eliminate weaknesses of an individual modality can offer improved diagnostics, therapeutic monitoring, and pre-clinical assessment of Fabry's disease. This review discusses the advantages and challenges in developing multimodality imaging systems of Fabry's cardiomyopathy, highlights some successful combinations that are now routinely used in the clinic and in research, and discusses recent advances in multimodality instrumentation that may offer new opportunities for pre-clinical assessment of this disease.

摘要

安德森法布里病(AFD)是一种罕见但诊断不足的细胞内脂质紊乱疾病,可导致左心室肥厚(LVH)。法布里病的临床前诊断很重要,因为它允许早期分层进行酶替代治疗,改善患者的长期预后,避免进展为不可逆的纤维化,并预防心血管并发症。整合每种成像方式的优势并同时消除单个成像方式弱点的成像方式组合,可以改善法布里病的诊断、治疗监测和临床前评估。本文综述了开发法布里心肌病多模态成像系统的优势和挑战,强调了一些目前在临床和研究中常规使用的成功组合,并讨论了多模态仪器的最新进展,这些进展可能为该疾病的临床前评估提供新的机会。

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