scholarly journals Assessment of Cardiac Involvement in Fabry Disease (FD) with Native T1 Mapping

2019 ◽  
Vol 191 (10) ◽  
pp. 932-939
Author(s):  
Fritz Christian Roller ◽  
Sven Fuest ◽  
Marco Meyer ◽  
Sebastian Harth ◽  
Dursun Gündüz ◽  
...  
Keyword(s):  
Fabry Disease ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Morphological Changes ◽  
Tissue Level ◽  
Therapeutic Benefit ◽  
Left Ventricular ◽  
Imaging Biomarker ◽  
Native T1 ◽  
Native T1 Time

Purpose Fabry disease (FD) is an X-linked multi-organ disorder of lysosomal metabolism with cardiac disease being the leading cause of death. Identifying early FD-specific pathologies is important in the context of maximum therapeutic benefit in these stages. Therefore, the aim of this study was to investigate the value of quantitative cardiac T1 mapping as a potential disease-specific surrogate. Methods 16 consecutive FD patients (9 female, 7 male; median age: 54 years, IQR 17) and 16 control patients (9 female, 7 male; median age: 52 years, IQR 20) were investigated at 1.5 Tesla. Native T1 mapping was performed using a modified look locker inversion recovery sequence (MOLLI) and native T1 times were measured within the septal myocardium at the midventricular short-axis section. Also functional parameters, left ventricular morphology, presence of late-gadolinium enhancement, cTnI- and Lyso-Gb3-Levels were evaluated. Results The median native septal T1 time for FD was 889.0 ms and 950.6 ms for controls (p < 0.003). LGE and positive cTnI values (0.26 ± 0.21) were present in 5 FD patients (31.25 %), and left ventricular hypertrophy (LVH) was present in 4 FD patients (25.00 %). The 4 cTnI and 8 Lyso-Gb3 positive FD patients had significantly lower native T1 values (p < 0.05, respectively p < 0.01). Assuming a T1 cut-off value of 900 ms for the identification of increased cardiac lipid deposit, 9 patients with FD (56.25 %) had pathologic values (4 patients cTnI and 8 patients Lyso-Gb3 positive). Moreover, native septal T1 showed a good negative correlation to Lyso-Gb3 (r = – 0.582; p = 0.018). Conclusion A pathologic cardiac native T1 time obviously reflects cardiac involvement in the scope of FD at tissue level. In the future native T1 mapping as an imaging biomarker might allow identification of early stages of cardiac involvement in FD before morphological changes are obvious. Key Points:  Citation Format

scholarly journals Effect of migalastat on cardiac involvement in Fabry disease: preliminary results from MAIORA study

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Camporeale ◽  
F Bandera ◽  
M Pieroni ◽  
F Pieruzzi ◽  
A Bersano ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Functional Capacity ◽  
Cardiac Involvement ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Real World Data ◽  
Private Company ◽  
Native T1 ◽  
Lv Mass ◽  
Treatment Naïve

Abstract Background Fabry Disease (FD) is a rare X-linked lysosomal storage disorder. Since 2016, pharmacological chaperone Migalastat has been approved for treatment of FD patients with amenable mutations to stabilize defective forms of the enzyme α-galactosidase A. A small but significant reduction in left ventricular (LV) mass after 18 months of Migalastat treatment has been previously reported by echocardiography. However, an integrated assessment of the effect of Migalastat on cardiac involvement, combining LV morphology and tissue composition by CMR with exercise capacity by cardiopulmonary test, is lacking. Purpose To determine the effects of 18 month treatment with Migalastat on LV mass, native T1 value and functional capacity in naïve patients with genetically confirmed FD cardiomyopathy. Methods Sixteen treatment naïve FD patients (4 females, mean age 46.4±16.2) with amenable mutations and signs of cardiac involvement underwent CMR with T1 mapping and cardio-pulmonary testing before and after 18 months of migalastat therapy as a part of MAIORA Study. Cardiac involvement was defined as presence of reduced native T1 values at CMR (a surrogate of myocardial glycosphingolipid storage) and/or LV hypertrophy (LVH). Nine patients (56%, 2 females, mean age 56.4±12.7 years) had LVH at baseline. Results Migalastat treatment was well tolerated in all patients, with no serious adverse event. No change in LV mass was detected at 18 months compared to baseline (95.2 (66.0–184.0) vs 103.0 (71.0–182.0) g/m2; p=0.5516). The same result was found after stratifying patients according to presence/absence of Late Gadolinium Enhancement (LGE) (LGE+ n=8, 2 females, mean age 56.2±13.1 years). There was a trend towards an increased native septal T1 value (870.0 (848–882) vs 860.0 (833.0–875.0) ms at baseline; p 0.056) with unchanged extracellular volume (ECV) (0.26 (0.23–0.028) vs 0.26 (0.22–0.29) at baseline; p 0.276) in the overall cohort. An improvement in functional capacity with a trend towards an increase in percent-predicted peak VO2 (72.0 (61.25–80.75) vs 67.0 (45.2–79.2) at baseline; p 0.056) and a significant increase in VO2 at anaerobic threshold (14.8 (12.6–20.0) vs 13.10 (6.8–18.6) ml/kg/min at baseline; p 0.004) was reported in the total population. Conclusion In treatment naïve FD patients with amenable mutations and signs of early or overt cardiac involvement, 18-month treatment with Migalastat stabilized LV mass both in patients with and without LGE and was associated with an improvement in exercise tolerance. The trend towards an increase in T1 value associated with unchanged ECV suggests partial clearance of cardiomyocyte glycoshingolipid storage. These real-world data are consistent with a beneficial impact of migalastat on the progression of cardiac involvement in FD. FUNDunding Acknowledgement Type of funding sources: Private company. Main funding source(s): Amicus Therapeutics

scholarly journals Myocardial native T1 mapping and correlations with clinical and CMR parameters in patients with systemic sclerosis

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Meloni ◽  
L Gargani ◽  
C Bruni ◽  
C Cavallaro ◽  
M Gobbo ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Myocardial Fibrosis ◽  
Healthy Subjects ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Left Ventricular ◽  
Volume Index ◽  
Imaging Protocol ◽  
Native T1 ◽  
Spin Echo Sequence

Abstract Funding Acknowledgements Type of funding sources: None. Background Systemic sclerosis (SSc) is a connective tissue disease characterized by diffuse vascular lesions and fibrosis, also affecting the heart. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis by late gadolinium enhancement (LGE) and interstitial myocardial fibrosis/edema by T1 mapping techniques. Purpose To evaluate the prevalence of cardiac involvement by native T1 mapping and its correlation with clinical and CMR parameters in SSc patients. Methods Fifty-one consecutive SSc patients (mean age 51.8 ± 13.7 years, 42 females) and 51 healthy subjects matched for age and sex underwent clinical, bio-humoral assessment, and CMR at 1.5T (Signa Artist, GE Healthcare ). The imaging protocol included: cine, T1 mapping by MOLLI, T2 mapping by multi-echo fast-spin-echo sequence, LGE, and STIR T2-weighted sequences. Native T1 and T2 values were assessed in all 16 myocardial segments and the global value was the mean. Results. Global native T1 values were significantly higher in SSc patients than in healthy subjects (1076.4 ± 50.7 vs 1033.3 ± 31.9 ms; P &lt; 0.0001). As in healthy subjects, in patients native T1 values were significantly lower in males than in females (1033.4 ± 38.3 vs 1085.6 ± 48.6 ms; P = 0.004) and inversely correlated with age (R=-0415; P = 0.002). Twenty-three (45.1%) patients had an increased global heart T1 value (&gt;1060 ms in males and &gt;1085 ms in females). Of them, 14 patients (60.9 %) showed positive LGE. Frequency of cardiovascular risk factors, indices of disease activity and chronicity, biochemical parameters, and cardio-active therapy were comparable between patients with normal and elevated T1. Compared to patients with normal T1 value, patients with elevated T1 had significantly higher left ventricular (LV) end-diastolic volume index (76.8 ± 13.3 vs 69.2 ± 11.8, P = 0.050), LV stroke volume index (49.7 ± 6.4 vs 44.4 ± 6.9 ml/m2; P = 0.010), LV cardiac output (3.6 ± 0.5  vs 3.0 ± 0.6 l/min /m2; P &lt; 0.0001), and global heart T2 values (60.1 ± 3.6 vs 55.7 ± 3.1 ms; P &lt; 0.0001). Replacement myocardial fibrosis was detected in 24 (47.1%) patients and they showed significantly higher global heart native T1 values (Figure 1A). Positive T2-weighted images for myocardial oedema were found in 5 (9.8%) patients, all with increased global heart native T1 value. Patients with oedema had significantly higher native global heart T1 values (Figure 1B). Conclusion Elevated native T1 values measured by CMR are frequent in SSc patients and they are associated with inflammation, replacement fibrosis, and increased LV dimension. CMR T1 mapping seems to be a sensitive parameter to include in the routine clinical assessment of SSc patients for detecting earlier pejorative cardiac involvement, although prospective data are recommended. Abstract Figure.

scholarly journals P1832 T1 mapping and echo-global longitudinal strain are early markers of cardiac involvement in patients with fabry disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Gatterer ◽  
D Beitzke ◽  
P E Bartko ◽  
M Schneider ◽  
T Binder ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Longitudinal Strain ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Global Longitudinal Strain ◽  
Lysosomal Storage Diseases ◽  
Left Ventricular ◽  
Study Cohort ◽  
Lysosomal Storage ◽  
Enzyme Replacement

Abstract OnBehalf Constantin Gatterer Introduction Fabry disease (FD) is one of the most common lysosomal storage diseases caused by deficiency of Alpha-Galactosidase A and accumulation of glycosphingolipids in all cells containing lysosomes. Cardiac involvement is characterized by left ventricular hypertrophy, conduction abnormalities and myocardial fibrosis with consecutive cardiomyopathy in late stages. Purpose As the detection of early signs of cardiac involvement is crucial for the initiation of enzyme replacement therapy (ERT) or Chaperon therapy, we intended to find early imaging markers. Methods Cardiac MRI (CMR) including Late Gadolinium Enhancement (LGE), representing fibrosis, and T1-mapping as well as echocardiography with measurement of global longitudinal strain (GLS) were done as part of the regular check-ups for patients with FD. Results The study cohort of 30 FD patients (20-69 years, 53% women) in different stages of disease showed low GLS values already at the time of baseline echocardiography (mean: -17,22%), correlating with the amount of LGE (r = 0,73; p = 0,003) and ejection fraction measured by CMR (CMR-EF; r=-0,74; p = 0,002). After an average follow-up of 39 months (STD 18), GLS values were significantly declined (-15,51 %; p = 0,009) and correlated with T1 times (r = 0,7; p = 0,04), while LGE and CMR-EF did not significantly change compared to baseline. Baseline T1 times correlated negatively with the reduction of GLS (r=-0,7; p = 0,003), while LGE and CMR-EF showed no correlation with the course of GLS. ERT appeared to have no influence on the extend of GLS reduction. Conclusion Low native CMR T1 times, indicating sphingolipid accumulation in the myocardium, seem to represent the beginning of cardiac involvement in FD and might predict the course of GLS. GLS is a sensitive parameter for early detection of cardiac manifestation and disease progression, while LGE and CMR-EF could not recognize slight deterioration of left ventricular function.

scholarly journals Value of Left Ventricular Feature Tracking Strain Analysis for Detection of Early Cardiac Involvement in Fabry Disease (FD)

2021 ◽  
Vol 10 (16) ◽  
pp. 3734
Author(s):  
Fritz Christian Roller ◽  
Alexander Brose ◽  
Martin Richter ◽  
Armin Schüssler ◽  
Sebastian Harth ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Feature Tracking ◽  
Large Scale ◽  
Cardiac Involvement ◽  
Radial Strain ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Control Subjects ◽  
Native T1 ◽  
Cardiac Manifestations

Purpose: Detection of cardiac involvement in Fabry disease (FD) is of high importance for treatment management. Native T1 mapping especially showed great potential for detection of early cardiac manifestations. Echocardiographic studies showed strain abnormalities in FD patients, but data on MRI feature tracking strain analysis (FT-SA) is limited. Therefore, the aim of our study was to evaluate the potential of FT-SA compared to native T1 and the FD specific biomarker Globotriaosylsphingosine (LysoGb3). Methods: 28 consecutive FD patients (18 female; 47.8 years ± 17.4 standard deviation (SD)) and 28 control subjects (18 female; 46.6 years ± 18.2 SD) underwent cardiac MRI at 1.5 Tesla. Global native T1 times and left ventricular FT-SA were evaluated. Results were correlated to serum Lyso-Gb3-levels. Results: Native T1 times, global longitudinal (GLS) and global radial strain (GRS) were significantly reduced in FD patients (p < 0.0064, p = 0.0009 and p = 0.0184, respectively). Moreover, native T1 times and GLS were significantly lower in Lyso-Gb3 positive FD patients (p < 0.005 and p = 0.03). GLS, native T1 times showed significant moderate correlations to LysoGb3 (p = 0.002 and p < 0.001). Furthermore, GLS and native T1 times reduce when LysoGb3 was elevated and increasingly with presence of left ventricular hypertrophy (LVH) or late gadolinium enhancement (LGE). Conclusions: Native T1 times and strain values differ significantly between FD patients and control subjects and showed promising correlations to the FD specific biomarker LysoGb3. We therefore conclude that strain abnormalities occur early beside native T1 reductions in cardiac FD involvement. Large scale trials are needed to verify our findings.

P5274Characterization of cardiac involvement in patients with MELAS syndrome in comparison to HCM patients by conventional LGE imaging and novel T1-mapping

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Bietenbeck ◽  
A R Florian ◽  
C Meier ◽  
V Holtstiege ◽  
G Chatzantonis ◽  
...  
Keyword(s):  
T1 Mapping ◽  
Cardiac Involvement ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Specific Pattern ◽  
Lv Function ◽  
Healthy Controls ◽  
Melas Syndrome ◽  
Native T1 ◽  
Lv Mass

Abstract Background MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a rare clinical subtype of mitochondrial myopathy. Cardiovascular magnetic resonance (CMR) images of MELAS-patients at an advanced state of the disease often show characteristic patterns: septal pronounced left ventricular (LV) hypertrophy and distinct focal myocardial scars in late-gadolinium-enhancement (LGE) images. This specific pattern is different from those seen in patients with hypertrophic cardiomyopathy (HCM) due to sarcomere protein mutations. Besides LGE imaging another method for the assessment of myocardial integrity, T1-mapping, has been established recently. This is the first study comparing native and enhanced T1-mapping (T1-na/en) as well as extracellular volume (ECV) values in HCM and MELAS. Methods 12 patients with confirmed MELAS syndrome at different states of the disease, 13 HCM patients and 15 controls were included. All CMR studies were performed on a 1.5T MR scanner (Ingenia, Philips) using bSSFP cine sequences for the assessment of LV-function and MOLLI sequences for T1-na/en mapping. 15min after IV injection of 0.1mmol/kg BW Gadobutrol for LGE imaging, T1-en maps were acquired. For comparison of groups T1-na/en and ECV values in a basal short axis slice were used. Results Median and interquartile ranges of LV function, T1 and ECV are shown in the table. There was no difference in LV ejection fraction (LVEF) between the groups, but end-diastolic LV-mass was increased in HCM- and MELAS-patients vs. controls (p<0.001, p=0.028). In MELAS-patients, myocardial fibrosis was detected in focal spots clearly defined in both septum and free lateral wall. In HCM patients there was a rather diffuse LGE pattern in the hypertrophic septum and the RV insertion points. Here T1-na/en and ECV significantly differed from those measured in healthy controls (p<0.001, p<0.001, p=0.002). In the MELAS group T1-na values were significantly higher compared to controls (p=0.004) and significantly lower compared to HCM patients (p=0.003). LV-function and mapping parameters of compared groups LV-EF, % LV-EDV, ml/m2 LV-mass, g/m2 T1-na, ms T1-en, ms ECV, % MELAS 57 (44–63) 86 (81–122) 80 (50–131) 969 (940–1033) 409 (381–465) 27 (24–35) HCM 60 (57–65) 50 (73–94) 94 (72–102) 1041 (1021–1074) 362 (346–430) 31 (28–36) Controls 61 (58–65) 72 (67–89) 53 (42–60) 937 (894–951) 464 (446–513) 26 (24–27) Conclusion Compared to healthy controls and HCM-patients, cardiac involvement in MELAS-patients is not only reliably visualized by conventional LGE imaging but also detected without the use of contrast agent by native T1-mapping. MELAS vs. HCM-patients show a different pattern of LGE and higher native T1 values, respectively.

Abstract 15137: Detection of Diffuse Myocardial Fibrosis using Multi-slice T1 Mapping by Slice Interleaved T1 (STONE) Sequence in Patients With Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Shingo Kato ◽  
Sébastien Roujol ◽  
Jihye Jang ◽  
Tamer Basha ◽  
Sophie Berg ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Myocardial Fibrosis ◽  
Wall Thickness ◽  
T1 Mapping ◽  
Free Breathing ◽  
Left Ventricular ◽  
Diffuse Myocardial Fibrosis ◽  
Mapping Technique ◽  
Native T1 ◽  
Native T1 Time

Introduction: In hypertrophic cardiomyopathy (HCM), there are significant variations in left ventricular (LV) wall thickness and fibrosis, which necessitates a volumetric coverage. Slice-interleaved T1 (STONE) mapping sequence allows for the assessment of native T1 time with complete coverage of LV myocardium. Hypothesis: We hypothesized that STONE sequence is useful for the assessment of regional native T1 time abnormality in HCM patients. Methods: Twenty-four septal HCM patients (56±16 years) and 10 healthy adult control subjects (57±15 years) were studied. Native T1 mapping was performed using STONE sequence which enables acquisition of 5 slices in the short-axis plane within a 90 sec free-breathing scan. We measured LV native T1 time and maximum LV wall thickness in each 16 segments from 3 slices (basal-, mid- and apical-slice) and evaluated the relationship between LV native T1 time and wall thickness. Late gadolinium enhanced (LGE) MRI was acquired to assess presence of myocardial enhancement. Results: In HCM patients, LV native T1 time was significantly elevated compared to healthy controls, regardless of presence or absence of LGE (mean native T1 time; LGE (+) segments (n=27), 1139±55 msec; LGE (-) segments (n=351), 1118±55 msec; healthy control (n=160),1065±35 msec; p<0.001 by one-way ANOVA, 6 segments were excluded from analysis due to artifacts). Among 351 segments without LGE, native LV T1 time was diffusely elevated over the 16 segments (Figure). Significant positive correlation was found between LV wall thickness and native LV T1 time (y=1013+8.7x, p<0.001). Conclusions: In HCM, substantial number of segments without LGE showed elevated native T1 time, and native T1 time was correlated with LV wall thickness. Multi-slice T1 mapping by using STONE sequence could be advantageous to overcome limited cardiac coverage of conventional single-slice T1 mapping technique and to accurately detect the diffuse myocardial fibrosis in HCM patients.

scholarly journals Left ventricular fibrosis and hypertrophy are associated with mortality in heart failure with preserved ejection fraction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pankaj Garg ◽  
Hosamadin Assadi ◽  
Rachel Jones ◽  
Wei Bin Chan ◽  
Peter Metherall ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
T1 Mapping ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Native T1 ◽  
Lv Mass ◽  
All Cause Mortality ◽  
Ventricular Fibrosis

AbstractCardiac magnetic resonance (CMR) is emerging as an important tool in the assessment of heart failure with preserved ejection fraction (HFpEF). This study sought to investigate the prognostic value of multiparametric CMR, including left and right heart volumetric assessment, native T1-mapping and LGE in HFpEF. In this retrospective study, we identified patients with HFpEF who have undergone CMR. CMR protocol included: cines, native T1-mapping and late gadolinium enhancement (LGE). The mean follow-up period was 3.2 ± 2.4 years. We identified 86 patients with HFpEF who had CMR. Of the 86 patients (85% hypertensive; 61% males; 14% cardiac amyloidosis), 27 (31%) patients died during the follow up period. From all the CMR metrics, LV mass (area under curve [AUC] 0.66, SE 0.07, 95% CI 0.54–0.76, p = 0.02), LGE fibrosis (AUC 0.59, SE 0.15, 95% CI 0.41–0.75, p = 0.03) and native T1-values (AUC 0.76, SE 0.09, 95% CI 0.58–0.88, p < 0.01) were the strongest predictors of all-cause mortality. The optimum thresholds for these were: LV mass > 133.24 g (hazard ratio [HR] 1.58, 95% CI 1.1–2.2, p < 0.01); LGE-fibrosis > 34.86% (HR 1.77, 95% CI 1.1–2.8, p = 0.01) and native T1 > 1056.42 ms (HR 2.36, 95% CI 0.9–6.4, p = 0.07). In multivariate cox regression, CMR score model comprising these three variables independently predicted mortality in HFpEF when compared to NTproBNP (HR 4 vs HR 1.65). In non-amyloid HFpEF cases, only native T1 > 1056.42 ms demonstrated higher mortality (AUC 0.833, p < 0.01). In patients with HFpEF, multiparametric CMR aids prognostication. Our results show that left ventricular fibrosis and hypertrophy quantified by CMR are associated with all-cause mortality in patients with HFpEF.

Cardiac magnetic resonance T1 mapping allows for predicting adverse left ventricular remodeling post-reperfused st-elevation myocardial infarction

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Zhang ◽  
Y.K Guo ◽  
Z.G Yang ◽  
M.X Yang ◽  
K.Y Diao ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
T1 Mapping ◽  
Ventricular Remodeling ◽  
Tissue Injury ◽  
Left Ventricular ◽  
Funding Source ◽  
Native T1 ◽  
End Diastolic Volume ◽  
Significant Difference ◽  
Lv Remodeling

Abstract Background Cardiac magnet resonance (CMR) T1 mapping allows the quantitative characterization of the severity of tissue injury and predict functional recovery in acute myocardial infarction (AMI). Purpose The study aimed to investigate whether native T1 and ECV of infarct myocardium are influenced by microvascular obstruction (MVO) and have predictive value for adverse left ventricular (LV) remodeling post-infarction. Method A cohort of 54 patients with successfully reperfused STEMI underwent CMR imaging at a 3T scanner in AMI and 3 months post-infarction. Native T1 data was acquired using a modified Look-Locker inversion recovery (MOLLI) sequence, and ECV maps were calculated using blood sampled hematocrit. Manual regions-of-interest were drawn within the infarct myocardium to measure native T1 and ECV (native T1infarct and ECVinfarct, respectively). MVO identified as a low-intensity area within the infarct zone on LGE was eliminated. Results MVO was present in 36 patients (66.67%) in AMI. ECVinfarct in patients with MVO was different from those without (58.66±8.71% vs. 49.64±8.82%, P=0.001), while no significant difference in T1infarct was observed between patients with and without MVO (1474.7±63.5ms vs. 1495.4±98.0ms, P=0.352). ECV correlated well with the change in end-diastolic volume (all patients: r=0.564, P&lt;0.001) and predicted LV remodeling in patients with and without MVO (rMVO absent = 0.626, P=0.005; rMVO present = 0.686, P&lt;0.001; all patients: r=0.622, P&lt;0.001); Native T1 was only associated with a 3-month change in LV end-diastolic volume (rMVO absent= 0.483, P=0.042) and predicted LV remodeling in patients without MVO (rMVO absent = 0.659, P=0.003). Furthermore, ECV had an association with LV remodeling (β=0.312, P=0.007) in multivariable logistic analysis. Conclusion Absolute native T1 in infarct myocardium might be affected by MVO but ECV isn't. ECV could predict LV remodeling in MI patients with and without MVO, while native T1 predict it in MI with MVO absent. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): 1·3·5 project for disciplines of excellence, West China Hospital, Sichuan University

scholarly journals Cardiovascular magnetic resonance-determined left ventricular myocardium impairment is associated with C-reactive protein and ST2 in patients with paroxysmal atrial fibrillation

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Lei Zhao ◽  
Songnan Li ◽  
Chen Zhang ◽  
Jie Tian ◽  
Aijia Lu ◽  
...  
Keyword(s):  
Cardiovascular Disease ◽  
Healthy Subjects ◽  
T1 Mapping ◽  
Circumferential Strain ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Healthy Controls ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Native T1

Abstract Background Myocardial strain assessed with cardiovascular magnetic resonance (CMR) feature tracking can detect early left ventricular (LV) myocardial deformation quantitatively in patients with a variety of cardiovascular diseases, but this method has not yet been applied to quantify myocardial strain in patients with atrial fibrillation (AF) and no coexistent cardiovascular disease, i.e., the early stage of AF. This study sought to compare LV myocardial strain and T1 mapping indices in AF patients and healthy subjects, and to investigate the associations of a portfolio of inflammation, cardiac remodeling and fibrosis biomarkers with LV myocardial strain and T1 mapping indices in AF patients with no coexistent cardiovascular disease. Methods The study consisted of 80 patients with paroxysmal AF patients and no coexistent cardiovascular disease and 20 age- and sex-matched healthy controls. Left atrial volume (LAV), LV myocardial strain and native T1 were assessed with CMR, and compared between the AF patients and healthy subjects. Biomarkers of C-reactive protein (CRP), transforming growth factor beta-1 (TGF-β1), collagen III N-terminal propeptide (PIIINP), and soluble suppression of tumorigenicity 2 (sST2) were obtained with blood tests, and compared between the AF patients and healthy controls. Associations of these biomarkers with those CMR-measured parameters were analyzed for the AF patients. Results For the CMR-measured parameters, the AF patients showed significantly larger LAV and LV end-systolic volume, and higher native T1 than the healthy controls (max P = 0.027). The absolute values of the LV peak systolic circumferential strain and its rate as well as the LV diastolic circumferential strain rate were all significantly reduced in the AF patients (all P < 0.001). For the biomarkers, the AF patients showed significantly larger CRP (an inflammation biomarker) and sST2 (a myocardium stiffness biomarker) than the controls (max P = 0.007). In the AF patients, the five CMR-measured parameters of LAV, three LV strain indices and native T1 were all significantly associated with these two biomarkers of CRP and sST2 (max P = 0.020). Conclusions In patients with paroxysmal AF and no coexistent cardiovascular disease, LAV enlargement and LV myocardium abnormalities were detected by CMR, and these abnormalities were associated with biomarkers that reflect inflammation and myocardial stiffness.

Proposed stages of Fabry disease: insights from multiparametric cardiac MRI and advanced ECG

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
JA Bicho Augusto ◽  
N Johner ◽  
D Shah ◽  
S Nordin ◽  
K Knott ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Cardiac Mri ◽  
Cardiac Involvement ◽  
T2 Mapping ◽  
Left Ventricular ◽  
P Wave ◽  
Stress Perfusion ◽  
Healthy Controls ◽  
T Wave ◽  
Advanced Analysis

Abstract Funding Acknowledgements Type of funding sources: None. Background Staging of Fabry disease (FD) cardiomyopathy uses multiparametric cardiac MRI. Advanced disease is characterized by left ventricular hypertrophy (LVH), myocardial inflammation/oedema (high native T2 mapping) and/or fibrosis (late gadolinium enhancement, LGE). Pre-LVH involvement has been described and includes myocardial sphingolipid storage (low native T1 mapping), impaired LV global longitudinal strain (GLS) and microvascular disease/dysfunction (low stress myocardial blood flow, MBF, in perfusion mapping). We aimed to define (1) the early myocardial phenotype prior to T1 lowering/pre-storage and (2) the stages of cardiac involvement in FD.   Methods FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, GLS, pre-contrast T1 and T2 mapping, adenosine stress perfusion mapping [for MBF] and LGE). Results 114 Fabry patients (46 ± 13 years, 61% female, 37% [n = 72] had LVH) and 76 controls (49 ± 15 years, 50% female) were included. FD with vs without LVH in brief and as expected, FD with LVH had significantly (p &lt; 0.05) lower MBF, GLS and T1, and higher T2 and %LGE. FD pre-LVH low T1 vs pre-LVH normal T1: low T1 patients (32/72, 44%) had higher LV mass index (67 ± 14 vs 59 ± 10g/m2, P = 0.011), maximum Q wave amplitude (2[1-2] vs 1[1-2]mm, P &lt; 0.001), Sokolow-Lyon index (22[16-28] vs 17[13-23]mm, P = 0.031) and more fractionated QRS complexes (44 vs 18%, P = 0.020). FD pre-LVH normal T1 vs healthy controls: normal T1 pre-LVH Fabry patients (40/72, 56%) had reduced GLS (-18 ± 2 vs -20 ± 2%, P &lt; 0.001), microvascular impairment (lower MBF 2.5 ± 0.7 vs 3.0 ± 0.8mL/g/min, P = 0.028), subtle T2 elevation (50 ± 4 vs 48 ± 2ms, p = 0.027) and limited LGE (%LGE 0.3 ± 1.1 vs 0%, P = 0.004) when compared to healthy controls; ECG abnormalities included shorter P wave duration (88 ± 12 vs 94 ± 15ms, P = 0.010) and T wave peak time (Tonset–Tpeak; 104 ± 28 vs 115 ± 20ms, P = 0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5 ± 0.4 vs 1.8 ± 0.4, P &lt; 0.001) compared to controls. Conclusion Prior staging of Fabry cardiomyopathy included a pre-LVH stage (accumulation/storage) and two LVH stages (hypertrophy and inflammation; fibrosis and impairment). Here we define an even earlier stage, pre-LVH pre-detectable storage, defined by microvascular dysfunction, impaired GLS and altered atrial depolarization and ventricular repolarization intervals (see Figure). Abstract Figure. Proposed stages of cardiac involvement

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