Assessment of Fabry variant (IVS4+919G>A) Cardiomyopathy in Taiwanese: Emphasis on T1 mapping

Assessment of Fabry variant (IVS4+919G>A) Cardiomyopathy in Taiwanese: Emphasis on T1 mapping

Background Fabry disease is a rare X-linked disorder characterized by deficiency ofa-galactosidase A, leading to progressive accumulation of glycosphingolipid in various organs, including the heart. Several studies have pointed out the unique pre-contrast T1 value character of classic Fabry cardiomyopathy, which is lower than the normal myocardium. In Taiwan, several recent studies pointed out ...

Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping.

BACKGROUND Anderson-Fabry disease (AFD) is a rare but underdiagnosed intracellular lipid disorder that can cause left ventricular hypertrophy (LVH). Lipid is known to shorten the magnetic resonance imaging parameter T1. We hypothesized that noncontrast T1 mapping by cardiovascular magnetic resonance would provide a novel and useful measure in this disease with potential to detect early cardiac ...

T1 mapping for characterization of myocardial fibrosis in hypertrophic cardiomyopathy

Background It was reported that the occurrence of myocardial fibrosis was related to sudden cardiac death and heart failure in patients with HCM. Late gadolinium enhancement (LGE) magnetic resonance imaging (MRI) is a widely used clinical method to detect fibrosis, however, its spatial resolution is limited. The purpose of this feasibility study is to determine the value of T1 mapping for the n...

Potential value of T1 mapping in cardiac MR assessment of hypertrophic cardiomyopathy and dilated cardiomyopathy patients: preliminary results

Background Myocardial fibrosis causes 1) increased stiffness and induces pathological signaling in cardiomyocytes resulting in progressive cardiac failure, 2)impairs mechanoelectric coupling of cardiomyocytes and increases the risk of arrhythmias. T1 mapping allow to quantify the total amount of myocardial extracellular volume and to subsequently define the amount of myocardial fibrosis. Quanti...

T1 Mapping with CMR Is Highly Sensitive for Fabry Disease Independent of Hypertrophy and Gender Thompson et al: T1 mapping with CMR in Fabry Disease