Journal Article

Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy

Hanneman, Kate, Iwanochko, Robert M., Morel, Chantal F., Wintersperger, Bernd J., Karur, Gauri R., Wasim, Syed, Mathur, Shobhit, Nguyen, Elsie T., Dreisbach, John G.

2019

Overview

Background Cardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. Methods In this prospective study, 38 FD patients (45.0 ± 14.5 years, 37% male) and 8 healthy controls (40.1 ± 13.7 years, 63% male) underwent 3 T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis. Results Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (− 15.3 ± 3.5% vs. − 16.3 ± 1.5%, p  = 0.45), GCS (− 19.4 ± 3.0% vs. -19.5 ± 2.9%, p  = 0.84) or base-to-apex LS gradient (7.5 ± 3.8% vs. 9.3 ± 3.5%, p  = 0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 ± 3.7% vs. 6.5 ± 2.2%, p  = 0.002) and native T1 (1170.2 ± 37.5 ms vs. 1239.0 ± 18.0 ms, p  < 0.001). Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy controls (OR 0.42, 95% CI: 0.20 to 0.86, p  = 0.019), even after controlling for native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p  = 0.049). In a nested logistic regression model with native T1, model fit was significantly improved by the addition of base-to-apex CS gradient (χ 2 (df = 1) = 11.04, p  < 0.001). Intra- and inter-observer agreement were moderate to good for myocardial strain parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). Conclusions CMR reproducibly identifies myocardial strain abnormalities in FD. Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in FD, with independent and incremental value beyond native T1.

• Publisher: BioMed Central,BioMed Central Ltd,Elsevier
• Subject: Adult / Angiology / Anopheles / Cardiology / Cardiomyopathies - diagnosis / Cardiomyopathies - physiopathology / Cardiomyopathy / Cardiovascular magnetic resonance / Case-Control Studies / Contrast Media - administration & dosage / Diagnosis / Diagnostic imaging / Fabry disease / Fabry Disease - diagnostic imaging / Fabry Disease - physiopathology / Fabry's disease / Female / Gadobutrol / Gadolinium / Heart hypertrophy / Humans / Hypertrophy / Hypertrophy, Left Ventricular - diagnosis / Hypertrophy, Left Ventricular - physiopathology / Imaging / Magnetic resonance imaging / Magnetic Resonance Imaging, Cine / Male / Medical research / Medicine / Medicine & Public Health / Middle Aged / Mortality / Myocardial Contraction / Myocardial diseases / Myocardial strain / Observer Variation / Organometallic Compounds - administration & dosage / Predictive Value of Tests / Prognosis / Prospective Studies / Radiology / Rare earth metal compounds / Reproducibility of Results / T1 mapping / Ventricular Function, Left / Ventricular Remodeling