Abstract:Background: Cardiac involvement is a major contributor of morbidity and mortality in Fabry disease (FD). Early detection and accurate evaluation of the disease progression is important in management.Cardiovascular magnetic resonance (CMR) derived feature fracking (FT) is a validated quantitative method of assessing myocardial deformation which may reflect early changes of myocardial function and track disease severity. We sought to evaluate the utility of CMR-FT as a measure of myocardial dysfunction in FD.Met… Show more
“…Then, each investigator blindly reviewed the full text of the remaining 22 articles, and 8 articles were excluded. The remaining 14 studies, including a total of 982 subjects (477 AFD patients and 505 control subjects), were the basis of the present meta-analysis [ 14 – 27 ]. Fig.…”
Section: Resultsmentioning
confidence: 99%
“…Characteristics and imaging data of the included studies are detailed in the Table 1 . Eleven studies were conducted prospectively [ 14 , 15 , 17 – 21 , 24 – 27 ] and 3 retrospectively [ 16 , 22 , 23 ]. Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ].…”
Section: Resultsmentioning
confidence: 99%
“…Eleven studies were conducted prospectively [ 14 , 15 , 17 – 21 , 24 – 27 ] and 3 retrospectively [ 16 , 22 , 23 ]. Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ]. Ten studies used 1.5T scanner [ 15 , 16 , 18 , 20 , 22 – 27 ] and 4 studies 3T scanner [ 14 , 17 , 19 , 21 ].…”
Section: Resultsmentioning
confidence: 99%
“…Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ]. Ten studies used 1.5T scanner [ 15 , 16 , 18 , 20 , 22 – 27 ] and 4 studies 3T scanner [ 14 , 17 , 19 , 21 ]. Demographic characteristics of the subjects included in the meta-analysis are reported in Table 2 .…”
Background
T1 mapping is an established cardiovascular magnetic resonance (CMR) technique that can characterize myocardial tissue. We aimed to determine the weighted mean native T1 values of Anderson-Fabry disease (AFD) patients and the standardized mean differences (SMD) as compared to healthy control subjects.
Methods
A comprehensive literature search of the PubMed, Scopus and Web of Science databases was conducted according to the PRISMA statement to retrieve original studies reporting myocardial native T1 values in AFD patients and healthy controls. A random effects model was used to calculate SMD, and meta-regression analysis was conducted to explore heterogeneity sources. Subgroup analysis was also performed according to scanner field strength and sequence type.
Results
From a total of 151 items, 14 articles were included in the final analysis accounting for a total population of 982 subjects. Overall, the weighted mean native T1 values was 984 ± 47 ms in AFD patients and 1016 ± 26 ms in controls (P < 0.0001) with a pooled SMD of − 2.38. In AFD patients there was an inverse correlation between native T1 values and male gender (P = 0.002) and left ventricular hypertrophy (LVH) (P < 0.001). Subgroup analyses confirmed lower T1 values in AFD patients compared to controls with a pooled SMD of − 2.54, − 2.28, − 2.46 for studies performed on 1.5T with modified Look-Locker inversion recovery (MOLLI), shortened MOLLI and saturation-recovery single-shot acquisition, respectively and of − 2.41 for studies conducted on 3T.
Conclusions
Our findings confirm a reduction of native T1 values in AFD patients compared to healthy controls and point out that the degree of T1 shortening in AFD is influenced by gender and LVH. Although T1 mapping is useful in proving cardiac involvement in AFD patients, there is need to standardize shreshold values according to imaging equipment and protocols.
“…Then, each investigator blindly reviewed the full text of the remaining 22 articles, and 8 articles were excluded. The remaining 14 studies, including a total of 982 subjects (477 AFD patients and 505 control subjects), were the basis of the present meta-analysis [ 14 – 27 ]. Fig.…”
Section: Resultsmentioning
confidence: 99%
“…Characteristics and imaging data of the included studies are detailed in the Table 1 . Eleven studies were conducted prospectively [ 14 , 15 , 17 – 21 , 24 – 27 ] and 3 retrospectively [ 16 , 22 , 23 ]. Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ].…”
Section: Resultsmentioning
confidence: 99%
“…Eleven studies were conducted prospectively [ 14 , 15 , 17 – 21 , 24 – 27 ] and 3 retrospectively [ 16 , 22 , 23 ]. Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ]. Ten studies used 1.5T scanner [ 15 , 16 , 18 , 20 , 22 – 27 ] and 4 studies 3T scanner [ 14 , 17 , 19 , 21 ].…”
Section: Resultsmentioning
confidence: 99%
“…Most of the investigations were based on single center experiences [ 14 – 17 , 19 – 27 ]. Ten studies used 1.5T scanner [ 15 , 16 , 18 , 20 , 22 – 27 ] and 4 studies 3T scanner [ 14 , 17 , 19 , 21 ]. Demographic characteristics of the subjects included in the meta-analysis are reported in Table 2 .…”
Background
T1 mapping is an established cardiovascular magnetic resonance (CMR) technique that can characterize myocardial tissue. We aimed to determine the weighted mean native T1 values of Anderson-Fabry disease (AFD) patients and the standardized mean differences (SMD) as compared to healthy control subjects.
Methods
A comprehensive literature search of the PubMed, Scopus and Web of Science databases was conducted according to the PRISMA statement to retrieve original studies reporting myocardial native T1 values in AFD patients and healthy controls. A random effects model was used to calculate SMD, and meta-regression analysis was conducted to explore heterogeneity sources. Subgroup analysis was also performed according to scanner field strength and sequence type.
Results
From a total of 151 items, 14 articles were included in the final analysis accounting for a total population of 982 subjects. Overall, the weighted mean native T1 values was 984 ± 47 ms in AFD patients and 1016 ± 26 ms in controls (P < 0.0001) with a pooled SMD of − 2.38. In AFD patients there was an inverse correlation between native T1 values and male gender (P = 0.002) and left ventricular hypertrophy (LVH) (P < 0.001). Subgroup analyses confirmed lower T1 values in AFD patients compared to controls with a pooled SMD of − 2.54, − 2.28, − 2.46 for studies performed on 1.5T with modified Look-Locker inversion recovery (MOLLI), shortened MOLLI and saturation-recovery single-shot acquisition, respectively and of − 2.41 for studies conducted on 3T.
Conclusions
Our findings confirm a reduction of native T1 values in AFD patients compared to healthy controls and point out that the degree of T1 shortening in AFD is influenced by gender and LVH. Although T1 mapping is useful in proving cardiac involvement in AFD patients, there is need to standardize shreshold values according to imaging equipment and protocols.
“…It is probably that compared with the volume of extracellular space, the volume of cardiomyocytes may have a greater impact on ECV under physiological conditions. Besides, it is potentially that CmyB might also be one of the influencing factors of ECV in diseased individuals, especially patients with altered sizes of cardiomyocytes, such as hypertrophic cardiomyopathy and Anderson-Fabry disease ( 49 , 50 ), which is currently unclear and needs to be confirmed by further research.…”
BackgroundThe extracellular volume fraction (ECV) derived from cardiac magnetic resonance imaging (MRI) is extensively used to evaluate myocardial fibrosis. However, due to the limited histological verification in healthy individuals, it remains unclear whether the size of cardiomyocytes may play a potential role in the physiological changes of ECV. The aim of this study was to examine the association between cardiomyocyte size and myocardial ECV by using a healthy porcine model.MethodsSixteen domestic healthy pigs were anesthetized and underwent cardiac MRI with mechanical controlled breathing. Intravenous contrast medium was introduced at a dose of 0.2–0.25 mmol/kg. The interventricular septum ECV was calculated using an established MRI procedure, which was based on the pre- and post-contrast T1 values of the heart and individual blood hematocrit. The cardiomyocyte breadth (CmyB) in cross section was measured by hematoxylin and eosin staining to reflect the cardiomyocyte size.ResultsData were successfully acquired from 14 pigs. The CmyB was obtained from the myocardial tissues corresponding to the region of interest on cardiac MRI. The mean ± SD of the ECV was 0.253 ± 0.043, and the mean ± SD of the CmyB was 10.02 ± 0.84 μm. The ECV exhibited a negative correlation with the CmyB (r = −0.729, p = 0.003).ConclusionThe myocardial ECV detected by cardiac MRI is negatively correlated with the CmyB in healthy pigs, demonstrating that the size of cardiomyocytes is potentially associated with the ECV under physiological conditions.
Feature-tracking cardiac magnetic resonance (FT-CMR), with the ability to quantify myocardial deformation, has a unique role in the evaluation of subclinical myocardial abnormalities. This review aimed to evaluate the clinical use of cardiac FT-CMR-based myocardial strain in patients with various systemic diseases with cardiac involvement, such as hypertension, diabetes, cancer-therapy-related toxicities, amyloidosis, systemic scleroderma, myopathies, rheumatoid arthritis, thalassemia major, and coronavirus disease 2019 (COVID-19). We concluded that FT-CMR-derived strain can improve the accuracy of risk stratification and predict cardiac outcomes in patients with systemic diseases prior to symptomatic cardiac dysfunction. Furthermore, FT-CMR is particularly useful for patients with diseases or conditions which are associated with subtle myocardial dysfunction that may not be accurately detected with traditional methods. Compared to patients with cardiovascular diseases, patients with systemic diseases are less likely to undergo regular cardiovascular imaging to detect cardiac defects, whereas cardiac involvement in these patients can lead to major adverse outcomes; hence, the importance of cardiac imaging modalities might be underestimated in this group of patients. In this review, we gathered currently available data on the newly introduced role of FT-CMR in the diagnosis and prognosis of various systemic conditions. Further research is needed to define reference values and establish the role of this sensitive imaging modality, as a robust marker in predicting outcomes across a wide spectrum of patients.
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