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

被引：5

作者：

Monda, Emanuele ^{[1
,2
]}

Falco, Luigi ^{[1
]}

Palmiero, Giuseppe ^{[1
]}

Rubino, Marta ^{[1
]}

Perna, Alessia ^{[1
]}

Diana, Gaetano ^{[1
]}

Verrillo, Federica ^{[1
]}

Dongiglio, Francesca ^{[1
]}

Cirillo, Annapaola ^{[1
]}

Fusco, Adelaide ^{[1
]}

Caiazza, Martina ^{[1
]}

Limongelli, Giuseppe ^{[1
,2
,3
]}

机构：

[1] Univ Campania Luigi Vanvitelli, Monaldi Hosp, Dept Translat Med Sci, Inherited & Rare Cardiovasc Dis, Naples, Italy

[2] UCL, Inst Cardiovasc Sci, London, England

[3] Univ Campania Luigi Vanvitelli, Inherited & Rare Cardiovasc Dis Clin, Dept Translat Med Sci, Monaldi Hosp,AORN Colli, Via L Bianchi 1, Naples, Italy

来源：

CARDIAC FAILURE REVIEW | 2023年 / 9卷

关键词：

Fabry's disease; hypertrophic cardiomyopathy; diagnosis; outcome; management; ENZYME-REPLACEMENT THERAPY; AGALSIDASE BETA TREATMENT; HUMAN ALPHA-GALACTOSIDASE; QUALITY-OF-LIFE; PEGUNIGALSIDASE ALPHA; SPECKLE-TRACKING; SAFETY; CARDIOMYOPATHIES; SOCIETY; PATIENT;

D O I：

10.15420/cfr.2023.06

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页数：7

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