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

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