Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future

被引：9

作者：

Esposito, Roberta ^{[1
,2
]}

Santoro, Ciro ^{[3
]}

Mandoli, Giulia Elena ^{[4
]}

Cuomo, Vittoria ^{[1
]}

Sorrentino, Regina ^{[3
]}

La Mura, Lucia ^{[3
]}

Pastore, Maria Concetta ^{[4
]}

Bandera, Francesco ^{[5
]}

D'Ascenzi, Flavio ^{[4
]}

Malagoli, Alessandro ^{[6
]}

Benfari, Giovanni ^{[7
]}

D'Andrea, Antonello ^{[8
]}

Cameli, Matteo ^{[4
]}

机构：

[1] Federico II Univ Hosp, Dept Clin Med & Surg, I-80122 Naples, Italy

[2] Mediterranea Cardioctr, I-80122 Naples, Italy

[3] Federico II Univ Hosp, Dept Adv Biomed Sci, I-80131 Naples, Italy

[4] Univ Siena, Dept Med Biotechnol, Div Cardiol, I-53100 Siena, Italy

[5] Univ Milan, IRCCS Policlin San Donato Milanese, Dept Cardiol, Heart Failure Unit, I-20122 Milan, Italy

[6] Univ Modena & Reggio Emilia, Baggiovara Hosp, Div Cardiol, Nephrocardiovasc Dept, I-41121 Modena, Italy

[7] Univ Verona, Dept Med, Div Cardiol, I-37132 Verona, Italy

[8] Luigi Vanvitelli Univ, Umberto Hosp Nocera Inferiore Salerno 1, Div Cardiol, I-81100 Caserta, Italy

来源：

JOURNAL OF CLINICAL MEDICINE | 2021年 / 10卷 / 09期

关键词：

Anderson-Fabry disease; echocardiography; cardiac involvement; cardiac imaging; multimodality imaging; CARDIOVASCULAR MAGNETIC-RESONANCE; LEFT-VENTRICULAR HYPERTROPHY; ENZYME-REPLACEMENT THERAPY; LATE GADOLINIUM ENHANCEMENT; CORONARY MICROVASCULAR DYSFUNCTION; SPECKLE-TRACKING; TISSUE DOPPLER; ECHOCARDIOGRAPHIC DETECTION; MYOCARDIAL FIBROSIS; ALPHA-GALACTOSIDASE;

D O I：

10.3390/jcm10091994

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease.

引用

页数：16

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