Cardiovascular Involvement in Fabry's Disease: New Advances in Diagnostic Strategies, Outcome Prediction and Management.

IF 4.2 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Cardiac Failure Review Pub Date : 2023-01-01 DOI:10.15420/cfr.2023.06

Emanuele Monda, Luigi Falco, Giuseppe Palmiero, Marta Rubino, Alessia Perna, Gaetano Diana, Federica Verrillo, Francesca Dongiglio, Annapaola Cirillo, Adelaide Fusco, Martina Caiazza, Giuseppe Limongelli

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Abstract

Cardiovascular involvement is common in Fabry's disease and is the leading cause of morbidity and mortality. The research is focused on identifying diagnostic clues suggestive of cardiovascular involvement in the preclinical stage of the disease through clinical and imaging markers. Different pathophysiologically driven therapies are currently or will soon be available for the treatment of Fabry's disease, with the most significant benefit observed in the early stages of the disease. Thus, early diagnosis and risk stratification for adverse outcomes are crucial to determine when to start an aetiological treatment. This review describes the cardiovascular involvement in Fabry's disease, focusing on the advances in diagnostic strategies, outcome prediction and disease management.

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法布里病的心血管介入:诊断策略、预后预测和管理的新进展

累及心血管是常见的法布里病，是发病率和死亡率的主要原因。该研究的重点是通过临床和影像学标志物识别提示心血管参与疾病临床前阶段的诊断线索。目前或即将有不同的病理生理驱动疗法可用于治疗法布里病，在疾病的早期阶段观察到最显著的益处。因此，早期诊断和不良后果的风险分层对于决定何时开始病原学治疗至关重要。本文综述了法布里氏病与心血管疾病的关系，重点介绍了诊断策略、预后预测和疾病管理方面的进展。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Cardiac Failure Review

CiteScore

5.60

自引率

0.00%

发文量

审稿时长

9 weeks