The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
| Autor: | Madiha Saiedi, Sanjana Patil, Anand N. Ganesan, Aleš Linhart, Joseph B. Selvanayagam, Ranjit Shah, Rebecca Perry |
|---|---|
| Přispěvatelé: | Perry, Rebecca, Shah, Ranjit, Saiedi, Madiha, Patil, Sanjana, Ganesan, Anand, Linhart, Ales, Selvanayagam, Joseph B |
| Rok vydání: | 2018 |
| Předmět: |
Male
medicine.medical_specialty Disease Anderson-Fabry disease 030204 cardiovascular system & hematology Left ventricular hypertrophy Ventricular Function Left 030218 nuclear medicine & medical imaging Diagnosis Differential cardiovascular magnetic resonance 03 medical and health sciences 0302 clinical medicine Predictive Value of Tests Internal medicine medicine echocardiography Humans Radiology Nuclear Medicine and imaging cardiovascular diseases Cardiac imaging Subclinical infection chaperone therapy medicine.diagnostic_test Ventricular Remodeling business.industry Myocardium Patient Selection Metabolic disorder Magnetic resonance imaging Enzyme replacement therapy medicine.disease Prognosis Fibrosis Magnetic Resonance Imaging Echocardiography Cardiology Fabry Disease Myocardial fibrosis Female Hypertrophy Left Ventricular Cardiology and Cardiovascular Medicine business enzyme replacement therapy |
| Zdroj: | JACC. Cardiovascular imaging. 12(7 Pt 1) |
| ISSN: | 1876-7591 |
| Popis: | Anderson-Fabry disease (AFD) is a rare X-linked inherited metabolic disorder which results in a deficiency or absence of the enzyme α-galactosidase A, leading to the accumulation of glycosphingolipids in various cells and organs including the heart. Cardiac involvement is common and results in increased myocardial inflammation, left ventricular hypertrophy (LVH), and myocardial fibrosis. Echocardiography and cardiovascular magnetic resonance (CMR) offer distinctive and often complementary use to assist in the diagnosis and monitoring pharmacologic therapy in AFD, including detection of the AFD cardiac phenotype, differentiation from other forms of LVH, and patient selection for therapeutic intervention. Advanced cardiac imaging holds promise in subclinical detection of AFD-related abnormalities as well as disease staging and prognostication. Refereed/Peer-reviewed |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|