scholarly journals In-depth phenotyping by cardiovascular magnetic resonance uncovering differences between ethnic groups in hypertensive heart disease

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A.T Mohamed ◽  
G Georgiopoulos ◽  
L Faconti ◽  
S Vennin ◽  
R McNally ◽  
...  

Abstract Background Black African/African-Caribbean individuals with hypertension (BH) are at greater risk of heart failure than those of white European ethnicity (WH). The mechanisms underlying this dissimilarity remain poorly understood. Purpose To investigate the influence of ethnicity on left ventricular (LV) remodelling using multi-parametric cardiovascular magnetic resonance (CMR). Methods BH (n=44), WH (n=38) and healthy-volunteers (HV; n=25, 5 of black ethnicity) underwent comprehensive CMR. The exam included: i) Arterial Stiffness/Afterload pulse-wave-velocity (PWV), aortic elastance (Ea) and systemic vascular resistance (SVR) by phase-contrast velocity-encoding imaging; ii) Ventricular remodelling/Function LV and right ventricular (RV) volumes, mass, ejection fraction (EF), LV peak-filling rate by short-axis cine images; myocardial strains were measured by feature tracking; iii) Left atrial (LA) remodelling/Function volumes and functions by long-axis cine images; iv) Tissue characterisation: extracellular volume by pre/post-contrast T1-mapping and late gadolinium enhancement (LGE) for interstitial and replacement myocardial fibrosis, respectively. Multivariate linear regression models were developed to investigate how LV remodelling associates with ethnicity, arterial afterload, including elastance (Ea) and stiffness [PW], and SVR. Models were adjusted for age, gender, body-mass-index, LV volumes or function and LA volumes. Results Subject characteristics are summarised in the Table. PWV and Ea and SVR were greater in hypertensives, particularly in BH, than HV; this was paralleled by higher LV mass, interventricular septum thickness (IVS), LA volumes but lower LV-EF. These findings were confirmed after adjusting for age. On the Model-1, IVS was associated with Ea (β=0.335, P=0.008) and black ethnicity (β=0.226, P=0.019) but not with SVR or PWV. For each increment of Ea there was a similar increase of IVS in BH and WH (P=0.602 for interaction), however BH had greater IVS than WH at each Ea value (Figure, fully-adjusted Model-1). On Model-2, LV end-diastolic volume was associated with Ea (β=−0.268, P=0.001), SVR (β=−0.319, P=0.019) but not with PWV or ethnicity. However, the inverse relation between LV size and Ea was significantly attenuated in BH (P=0.039 for interaction), (Figure, fully-adjusted Model-2). On model-3, LV-EF was associated with Ea (β=0.223, P=0.009) but not with ethnicity, PWV or SVR. LV-EF reduction for each Ea increment was similar for BH and WH (P=0.597 for interaction). Conclusion BH and WH show a distinctive LV remodelling phenotype. BH had a greater susceptibility to hypertrophy and an attenuated reduction of chamber size in response to arterial afterload. Further research to disentangle the genetic and environmental factors underlying these ethnic group-specific differences is utterly required. FUNDunding Acknowledgement Type of funding sources: None. Figure 1 Table 1

2016 ◽  
Vol 20 (2) ◽  
Author(s):  
Rebecca Schofield ◽  
Katia Manacho ◽  
Silvia Castelletti ◽  
James C. Moon

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. Cardiac imaging plays a key role in the diagnosis and management, with cardiovascular magnetic resonance (CMR) an important modality. CMR provides a number of different techniques in one examination: structure and function, flow imaging and tissue characterisation particularly with the late gadolinium enhancement (LGE) technique. Other techniques include vasodilator perfusion, mapping (especially T1 mapping and extracellular volume quantification [ECV]) and diffusion-weighted imaging with its potential to detect disarray. Clinically, the uses of CMR are diverse. The imaging must be considered within the context of work-up, particularly the personal and family history, Electrocardiogram (ECG) and echocardiogram findings. Subtle markers of possible HCM can be identified in genotype positive left ventricular hypertrophy (LVH)-negative subjects. CMR has particular advantages for assessment of the left ventricle (LV) apex and is able to detect both missed LVH (apical and basal antero-septum), when the echocardiography is normal but the ECG abnormal. CMR is important in distinguishing HCM from both common phenocopies (hypertensive heart disease, athletic adaptation, ageing related changes) and rarer pheno and/or genocopies such as Fabry disease and amyloidosis. For these, in particular the LGE technique and T1 mapping are very useful with a low T1 in Fabry’s, and high T1 and very high ECV in amyloidosis. Moreover, the tissue characterisation that is possible using CMR offers a potential role in patient risk stratification, as scar is a very strong predictor of future heart failure. Scar may also play a role in the prediction of sudden death. CMR is helpful in follow-up assessment, especially after septal alcohol ablation and myomectomy.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Haotian Gu ◽  
Rong Bing ◽  
Calvin Chin ◽  
Lingyun Fang ◽  
Audrey C. White ◽  
...  

Abstract Background First-phase ejection fraction (EF1; the ejection fraction measured during active systole up to the time of maximal aortic flow) measured by transthoracic echocardiography (TTE) is a powerful predictor of outcomes in patients with aortic stenosis. We aimed to assess whether cardiovascular magnetic resonance (CMR) might provide more precise measurements of EF1 than TTE and to examine the correlation of CMR EF1 with measures of fibrosis. Methods In 141 patients with at least mild aortic stenosis, we measured CMR EF1 from a short-axis 3D stack and compared its variability with TTE EF1, and its associations with myocardial fibrosis and clinical outcome (aortic valve replacement (AVR) or death). Results Intra- and inter-observer variation of CMR EF1 (standard deviations of differences within and between observers of 2.3% and 2.5% units respectively) was approximately 50% that of TTE EF1. CMR EF1 was strongly predictive of AVR or death. On multivariable Cox proportional hazards analysis, the hazard ratio for CMR EF1 was 0.93 (95% confidence interval 0.89–0.97, p = 0.001) per % change in EF1 and, apart from aortic valve gradient, CMR EF1 was the only imaging or biochemical measure independently predictive of outcome. Indexed extracellular volume was associated with AVR or death, but not after adjusting for EF1. Conclusions EF1 is a simple robust marker of early left ventricular impairment that can be precisely measured by CMR and predicts outcome in aortic stenosis. Its measurement by CMR is more reproducible than that by TTE and may facilitate left ventricular structure–function analysis.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Elizabeth W. Thompson ◽  
Srikant Kamesh Iyer ◽  
Michael P. Solomon ◽  
Zhaohuan Li ◽  
Qiang Zhang ◽  
...  

Abstract Background Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular wall thickness, cardiomyocyte hypertrophy, and fibrosis. Adverse cardiac risk characterization has been performed using late gadolinium enhancement (LGE), native T1, and extracellular volume (ECV). Relaxation time constants are affected by background field inhomogeneity. T1ρ utilizes a spin-lock pulse to decrease the effect of unwanted relaxation. The objective of this study was to study T1ρ as compared to T1, ECV, and LGE in HCM patients. Methods HCM patients were recruited as part of the Novel Markers of Prognosis in Hypertrophic Cardiomyopathy study, and healthy controls were matched for comparison. In addition to cardiac functional imaging, subjects underwent T1 and T1ρ cardiovascular magnetic resonance imaging at short-axis positions at 1.5T. Subjects received gadolinium and underwent LGE imaging 15–20 min after injection covering the entire heart. Corresponding basal and mid short axis LGE slices were selected for comparison with T1 and T1ρ. Full-width half-maximum thresholding was used to determine the percent enhancement area in each LGE-positive slice by LGE, T1, and T1ρ. Two clinicians independently reviewed LGE images for presence or absence of enhancement. If in agreement, the image was labeled positive (LGE + +) or negative (LGE −−); otherwise, the image was labeled equivocal (LGE + −). Results In 40 HCM patients and 10 controls, T1 percent enhancement area (Spearman’s rho = 0.61, p < 1e-5) and T1ρ percent enhancement area (Spearman’s rho = 0.48, p < 0.001e-3) correlated with LGE percent enhancement area. T1 and T1ρ percent enhancement areas were also correlated (Spearman’s rho = 0.28, p = 0.047). For both T1 and T1ρ, HCM patients demonstrated significantly longer relaxation times compared to controls in each LGE category (p < 0.001 for all). HCM patients also showed significantly higher ECV compared to controls in each LGE category (p < 0.01 for all), and LGE −− slices had lower ECV than LGE + + (p = 0.01). Conclusions Hyperenhancement areas as measured by T1ρ and LGE are moderately correlated. T1, T1ρ, and ECV were elevated in HCM patients compared to controls, irrespective of the presence of LGE. These findings warrant additional studies to investigate the prognostic utility of T1ρ imaging in the evaluation of HCM patients.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Alderighi ◽  
A Baritussio ◽  
O Ozden Tok ◽  
M Perazzolo Marra ◽  
S Iliceto ◽  
...  

Abstract Background Clinically manifest cardiac sarcoidosis (CS) has a prevalence of 5%, but is more frequent in autoptic series (25%). Diagnosis is multiparametric and relies on clinical criteria and imaging findings, although a certain diagnosis, especially in the case of isolated CS (ICS), can only be based on endomyocardial biopsy. Cardiovascular magnetic resonance (CMR) has a comprehensive role in the assessment of CS: left ventricular (LV) dysfunction and extent of late gadolinium enhancement (LGE)are important predictors of prognosis, T2 mapping provides information on disease activity and global longitudinal strain (GLS) analysis can uncover subclinical LV impairment. Purpose To assess the prevalence of CS by CMR in patients with biopsy-proven extracardiacsarcoidosis (ECS); to describe the imaging characteristics of patients with ECS and those with high clinical suspicionof ICS; to investigate the contribution of more recent techniques to the diagnosis of CS alongside traditional LGE assessment. Methods We retrospectively enrolled 84 patients (66% males, mean age 59±13 years) referred to our centreforsuspected CS (biopsy-proven ECS, n=61; clinical presentation suggestive of CS,, n=23). CMR was performed on a 1.5T scanner, with a protocol comprehensive of biventricular functional assessment and post-contrast images; T2-STIR images (n=30), native myocardial T1 mapping (n=24) and T2 mapping (n=19) were also performed in selected patients. Tissue tracking analysis was perfomed in all patients using a dedicated software. Results Based on CMR findings, 35 patients (42%) with ECS did not show cardiac involvement (SS), 26 (31%) showed both cardiac and systemic involvement (CS-SS) and 23 (27%) had evidence of ICS (ICS). 43% of patients had history of arrhythmias, but life-threatening tachyarrhythmiaswere more frequent in patients with CS (p=0.02).Patients with CS had significantly lower LVEF (p&lt;0,01), larger LV volumes (p&lt;0,01) and greater LV mass (p&lt;0,01). GLS values were impaired in all the groups but significantly more in patients with CS (p&lt;0,01). With regards to LGE distribution, ICS patients showed a higher number of segments involved (p=0,011) as compared to CS patients. T2-STIRimages were positive in 3 out of 30 patients; T2 mapping detected myocardial oedema in 1 patient with negative T2- STIR and was positive in 7 who did not undergo traditional oedema evaluation. T1 mapping mainly confirmed the results provided by LGE, but was altered in 1 patient who could not receive gadolinium. Conclusions CMR findings consistent with CS were found in 49 patients referred for suspected CS. Patients with cardiac involvement, particularly if isolated, had significantly lower LVEF, greater LV volumes and more impaired GLS. Patients with SS, despite a normal LV function, showed mildly impaired GLS, subtending subclinical cardiac involvement. Funding Acknowledgement Type of funding source: None


2020 ◽  
Author(s):  
Shanat Baig ◽  
Rory Dowd ◽  
Nicola C Edwards ◽  
James Hodson ◽  
Larissa Fabritz ◽  
...  

Abstract Background Alström syndrome (ALMS) is a rare ciliopathy characterised by early onset insulin resistance, obesity, and dyslipidaemia and is a model for diseases that have huge social, health and economic impact. Cardiomyopathy develops in the majority, with high rates of morbidity and mortality, the definitive feature of which is the presence of coarse replacement fibrosis and diffuse myocardial fibrosis (DIF). The pathogenesis of heart failure is thought to involve fibroblast accumulation and expansion of the extracellular matrix with excess protein deposition, leading to distorted organ architecture and impaired contractile function. Consecutive adults with genetically proven ALMS attending the National Centre for Rare Disease in Birmingham, England were studied. All patients underwent serial CMR, echocardiography and venous blood sampling following standard clinical practice, with computed tomography coronary angiography (CTCA) performed to assess severity of CAD. The aims of this study were: 1) to evaluate changes over time in DIF by cardiovascular magnetic resonance tissue characterization in ALMS; 2) to examine whether changes in DIF are associated with alteration in systolic or diastolic function; and 3) to evaluate the frequency and severity of coronary artery disease as a confounder for progression of ischaemic versus non-ischaemic fibrosis. Results In total, 30/32 adults (63% male; 67% White British) participated. The median age at first scan was 21.3 years (interquartile range: 19.0-32.6) and participants were followed for a maximum of 67 months. Only 4 patients had significant coronary artery stenosis on post-mortem, invasive or CTCA. Mid short axis myocardial T1 times, myocardial extracellular volume, and left ventricular mass increased significantly over time, by an average of 21.8ms (95% CI 17.4 – 26.1; p<0.001), 0.011 (0.006 – 0.016, p<0.001), and 2.8 g/m 2 (1.9 – 3.7; p<0.001) per year, respectively. These changes were not associated with significant deterioration in myocardial structure or function. Conclusions This is the first comprehensive prospective study demonstrating progression of DIF in ALMS over time, although no structural or functional consequences were noted within a median three and a half years’ follow up. Further study is warranted to define whether DIF is a by-stander or the driver to impaired contractile function, heart failure and death.


2015 ◽  
Vol 16 (suppl 2) ◽  
pp. S150-S152 ◽  
Author(s):  
P Bazal ◽  
O-A Nastase ◽  
MS Vieira ◽  
A M Maceira Gonzalez ◽  
J Kowal ◽  
...  

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Xincheng Jiang ◽  
Yuanwei Xu ◽  
Jiayu Sun ◽  
Lili Wang ◽  
Xinli Guo ◽  
...  

Abstract Phospholamban (PLN) is an important regulator for sarcoendoplasmic reticulum (SR) calcium transport ATPase (SERCA), which uptakes Ca2+ to SR during the diastolic phase of cardiomyocytes to maintain intracellular calcium homeostasis. Mutations on PLN result in intracellular calcium disorder, myocardial contraction defect, and eventually heart failure and/or malignant ventricular arrhythmia. Since 2003, several kinds of PLN mutations have been identified in familial dilated cardiomyopathy (DCM) patients, illustrating a few clinical characteristics that differs from classical DCM patients. Herein, we report a large PLN-R14del family with typical clinical characteristics reported including relatively late-onset clinical symptoms, low-voltage in ECG, as well as frequent ventricular arrythmias. Moreover, members underwent cardiac magnetic resonance (CMR) examination showed a strikingly similar pattern of late gadolinium enhancement (LGE)—Sub-epicardial involvement in the left ventricular (LV) lateral wall with or without linear mid-wall enhancement in the interventricular septum. The former one can also present in younger PLN-R14del carriers despite completely normal LV structure and function. Meanwhile, T1 mapping also found significantly increased extracellular volume (ECV) in PLN-R14del carriers. These findings highlight the special role of CMR to phenotyping PLN-induced cardiomyopathy patients and distinguish them from other types of cardiomyopathy.


2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Yongning Shang ◽  
Xiaochun Zhang ◽  
Liu Chen ◽  
Weiling Leng ◽  
Xiaotian Lei ◽  
...  

Background. Diabetic cardiomyopathy (DCM) is always accompanied with alteration of left ventricular structure and function. The aims of this study were to assess the structural remodelling in patients with DCM by cardiovascular magnetic resonance (CMR) and correlation of structural remodelling with severity of DCM.Methods. Twenty-five patients (53.8±8.8years, 52.0% males) with DCM and thirty-one normal healthy controls (51.9±13.6years, 45.2% males) were scanned by CMR cine to assess function and structure of left ventricular. Length of diabetic history and results of cardiac echocardiography (E′, A′, and E′/A′) were also measured.Results. Compared with normal controls group, DCM group was associated with significantly increased ratio of left ventricular mass at end diastole to end-diastolic volume (MVR) (P<0.05) and no significant difference was in mass at end diastole (P>0.05). The ratio correlated with both length of diabetic history and echocardiographic Doppler tissue imaging E′ (allP<0.05).Conclusions. CMR can be a powerful technique to assess LV remodelling, and MVR may be considered as an imaging marker to evaluate the severity of LV remodelling in patients with DCM.


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