磁共振成像2023，Vol.14Issue(12)：172-176,5.DOI:10.12015/issn.1674-8034.2023.12.031
法布雷病心脏磁共振应用进展
Advances in the application of cardiac MRI in diagnosis of Fabry disease
摘要
Abstract
Fabry disease is a rare X-linked genetic lysosomal storage disorder,which leads to reduction of α-galactosidase A and accumulation of metabolic substrates in heart and other organs.Cardiac magnetic resonance(CMR)with development of new techniques,such as mapping analysis,late gadolinium enhancement(LGE)assessment,strain imaging and perfusion imaging,plays an important role in distinguishing FD from other hypertrophic heart conditions.CMR offers a comprehensive evaluation of cardiac structure,function,and histological characteristics in a'one-stop shop'approach.Our study comprehensively reviewed the characteristic features of FD in various CMR techniques,including tissue changes in the early stages of the disease.Furthermore,we emphasized the significant role of CMR in early diagnosis,risk stratification,prognosis assessment,and treatment monitoring in FD.The aim is to achieve early and accurate diagnosis of FD in the clinical practice,differentiate FD from other hypertrophic cardiac diseases,thus enabling early intervention and treatment for FD patients,ultimately improving prognosis.关键词
法布雷病/心脏磁共振/磁共振成像/T1 mapping/左心室肥厚/心肌病Key words
Fabry disease/cardiac magnetic resonance/magnetic resonance imaging/T1 mapping/left ventricular hypertrophy/cardiomyopathy分类
医药卫生引用本文复制引用
李裕国,赵韧,余永强,侯唯姝,李小虎..法布雷病心脏磁共振应用进展[J].磁共振成像,2023,14(12):172-176,5.基金项目
National Natural Science Foundation of China(No.82071897). 国家自然科学基金项目(编号:82071897) （No.82071897）
