scholarly journals Incremental value of cardiovascular magnetic resonance imaging in family screening for hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
R Huurman ◽  
N Van Der Velde ◽  
H Hassing ◽  
R Budde ◽  
M Van Slegtenhorst ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Genetic testing in relatives of hypertrophic cardiomyopathy (HCM) patients can lead to early identification of carriers of pathogenic DNA variants (G+), before onset of left ventricular hypertrophy (LVH). Repeated evaluation by electrocardiography (ECG) and transthoracic echocardiography (TTE) is recommended to detect HCM during follow-up. Cardiovascular magnetic resonance (CMR) imaging has become valuable in the work-up of HCM, although its role in G+ subjects has not been extensively evaluated. In this study, we investigated the value of CMR in the G+/LVH- population. We included 55 G+ subjects who underwent CMR in addition to ECG and TTE, with a maximal wall thickness (MWT) <15mm on TTE. The CMR imaging protocol consisted at least of steady state free procession imaging and 2-dimensional late gadolinium enhancement (LGE) images. ECGs were considered abnormal in case of pathologic Q waves, T wave inversion or signs of LVH (by voltage criteria including Sokolow-Lyon and a Romhilt-Estes score ≥4). TTEs were abnormal in case of LVH (defined as MWT≥10mm). For both modalities, the diagnosis of HCM was based on a MWT≥13mm. The yield of CMR relative to ECG/TTE was assessed by comparing the proportion of HCM diagnoses and the presence of other phenotypic features. Forward step logistic regression was used to assess whether the presence of TTE/ECG abnormalities could predict reclassifications or abnormalities (crypts and LGE) on CMR. An overview of ECG/TTE and CMR findings is shown in the Figure. Two of 16 (13%) subjects diagnosed with HCM on TTE were reclassified as having no HCM on CMR, and 8 of 39 (21%) subjects without HCM on TTE were reclassified as HCM on CMR. These 8 subjects had a mean MWT of 15.4 ± 2.6 mm on CMR and a mean MWT difference of 4.5 ± 2.9 mm (range 1.7-9.4) compared to TTE, which in 3 cases was explained by a hook-shaped thickening of the basal anterior wall in the 2 chamber view, not visible on TTE. Compared to subjects without HCM on both modalities, the reclassified group had a significantly higher QRS duration (104 ± 14 vs 93 ± 11 ms, p = 0.03) and anterior mitral valve leaflet length (30 ± 4 vs 26 ± 3 mm, p = 0.01). Of the 13 subjects with normal ECG/TTE results, none were reclassified as HCM using CMR. The proportion of additional CMR abnormalities was large in subjects with and without abnormal ECG/TTE results (57% vs 38%, p = 0.24). Subjects with poor TTE image quality were equally likely to be reclassified compared to those with sufficient image quality (10% vs 24%, p = 0.19). Logistic regression demonstrated that the presence of TTE/ECG abnormalities (odds ratio [OR] 8.7 [1.3-59.0], p = 0.03) and age (OR 1.1 [1.0-1.2], p < 0.01) independently predicted reclassifications or presence of abnormalities using CMR. Additional CMR imaging reclassifies 18% of subjects. Subjects with normal ECG and TTE results are not diagnosed as HCM on CMR, but the prevalence of HCM-related abnormalities on CMR was high in subjects with and without ECG/TTE abnormalities. Abstract Figure. Diagnostic approach and CMR findings

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Sopova ◽  
C Park ◽  
A Al-Atta ◽  
K Bennaceur ◽  
A Mohammad ◽  
...  

Abstract Background Adverse left ventricular (LV) remodelling is associated with development of heart failure and poor outcomes in patients with acute myocardial infarction (AMI). Understanding the immunomodulatory mechanisms of LV remodelling is an essential step for the development of novel therapies. Interferon-γ-inducible protein-10 (IP-10)/CXCL10 is a chemokine involved in the recruitment of activated T cells into sites of tissue inflammation. Although IP-10 was reported to reduce adverse LV remodeling in a preclinical myocardial infarction model, its role in LV remodeling in humans with AMI remains unknown. Purpose To determine the clinical predictive value of serum IP-10 in LV remodeling in patients with ST-segment elevation myocardial infarction (STEMI). Methods This is a substudy of the double-blind, randomised controlled trial “Evaluating the effectiveness of intravenous ciclosporin on reducing reperfusion injury in patients undergoing primary percutaneous coronary intervention” (CAPRI; ClinicalTrials.gov registry number NCT02390674), which enrolled 52 acute STEMI patients. LV remodeling was assessed by cardiovascular magnetic resonance (CMR) imaging and was defined as the 12-week vs. the 3-day post-myocardial infarction change of the left ventricular ejection fraction (ΔLVEF), LV end-diastolic volume (ΔEDV) or LV end-systolic volume (ΔESV). Serum IP-10 was measured before and 5min, 15min, 30min, 90min and 24h after reperfusion by ELISA. Linear regression analysis was used to determine the independent association of IP-10 with the endpoints of the study. Results Serum IP-10 concentration peaked at 30min after reperfusion followed by a 2-fold decrease at the 24h post reperfusion compared to pre-reperfusion levels (P<0.001 for all). Comparison of the 12-week CMR to the baseline CMR imaging revealed that baseline pre-reperfusion as well as 5min, 15min, 30min and 90min, but not 24h, post-reperfusion IP-10 serum levels associated with increased LVEF and decreased ESV at 12-weeks (range correlation coefficient r=[0.35–0.41], P<0.05 with ΔLVEF and r=[−0.33 to −0.44], P<0.05 with ΔESV) indicating that the increase of IP-10 at the acute phase of myocardial infarction confers a cardioprotective role. Multivariable linear regression analysis for ΔLVEF showed that in a model including baseline pre-reperfusion or 5min or 15min or 30min or 90min post-reperfusion IP-10 and age, gender, traditional risk factors (arterial hypertension, body-mass index, hyperlipoproteinemia, diabetes mellitus, smoking, family history of CAD), infarct location, admission high-sensitivity troponin T, door-to-balloon time and ciclosporin treatment, only IP-10 was the independent determinant of ΔLVEF. Conclusions Increased serum IP-10 levels early after reperfusion are associated with reverse LV remodeling in patients with STEMI undergoing primary PCI. The clinical application of IP-10 as a novel biomarker of LV remodeling post-AMI should be further explored and validated. Funding Acknowledgement Type of funding source: None


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.


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 42 (Supplement_1) ◽  
Author(s):  
V Puntmann ◽  
S Martin ◽  
B Vanchin ◽  
N Holm ◽  
E Giokoglu ◽  
...  

Abstract Background Long COVID (LC) is an increasingly recognized late complication of COVID-19 infection. Cardiovascular involvement has also been implicated, however, the type and extent of the underlying cardiovascular injury remains unknown. Purpose To evaluate the association between ongoing symptoms and findings with cardiovascular magnetic resonance (CMR) in consecutive patients recently recovered from COVID-19 illness. Methods Prospective observational cohort study of patients recently recovered from COVID-19 illness and no previously known cardiovascular disease were included between April 2020 and April 2021. Demographic characteristics, cardiac blood markers, and CMR imaging a minimum of 4 weeks from the diagnosis were obtained. Results Of the 389 included patients, 192 (49%) were male, the mean (±standard deviation) age was 44 (±13) years and 61 (16%) required hospitalization during the acute illness. The median (IQR) time interval between COVID-19 diagnosis and CMR was 94 (71–165) days. 298 (77%) of patients continued to experience ongoing cardiovascular symptoms (long COVID, LC), including dyspnea, palpitations, atypical chest pain and fatigue at the time of CMR at least 4 weeks after the infection. In most patients, the symptoms were only effort related 137 (46%), whereas in 98 (33%) the symptoms affected the activities of daily life; 10 (3%) had severe and debilitating symptoms at rest. Compared to those with no LC (NLC, n=91), LC patients were more commonly hospitalized, had significantly higher native T1, native T2, and showed pericardial enhancement and effusion (Figure 1). There were no differences in cardiac biomarkers, left ventricular (LV) and right ventricular ejection fraction and mass. Proportionally, men and women were similarly affected (n=144 (73%) vs. n=157 (80%), p=0.18). Previous hospitalization was associated with hypertension and ongoing detectable troponin. LC status was associated with previous hospitalization and CMR findings of raised native T1 and native T2, and in females also pericardial enhancement. Severity of symptoms were associated with increased native T1 and T2 and decreased end-diastolic volume, whereas cardiac function showed no significant difference. Conclusions In this cohort of patients recently recovered from COVID-19 infection, ongoing cardiovascular symptoms were common. The LC status was related to previous hospitalization and CMR imaging findings of myopericardial inflammation. The extent and type of cardiovascular findings was associated with the severity of symptoms. These findings indicate the need for ongoing investigation of the long-term cardiovascular consequences of COVID-19. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The German Heart Foundation (Deutsche Herzstiftung) and Bayer AG, Leverkusen, Germany Figure 1


Author(s):  
Qiang Zhang ◽  
Matthew K. Burrage ◽  
Elena Lukaschuk ◽  
Mayooran Shanmuganathan ◽  
Iulia A. Popescu ◽  
...  

Background: Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging is the gold standard for non-invasive myocardial tissue characterization, but requires intravenous contrast agent administration. It is highly desired to develop a contrast-agent-free technology to replace LGE for faster and cheaper CMR scans. Methods: A CMR Virtual Native Enhancement (VNE) imaging technology was developed using artificial intelligence. The deep learning model for generating VNE uses multiple streams of convolutional neural networks to exploit and enhance the existing signals in native T1-maps (pixel-wise maps of tissue T1 relaxation times) and cine imaging of cardiac structure and function, presenting them as LGE-equivalent images. The VNE generator was trained using generative adversarial networks. This technology was first developed on CMR datasets from the multi-center Hypertrophic Cardiomyopathy Registry (HCMR), using HCM as an exemplar. The datasets were randomized into two independent groups for deep learning training and testing. The test data of VNE and LGE were scored and contoured by experienced human operators to assess image quality, visuospatial agreement and myocardial lesion burden quantification. Image quality was compared using nonparametric Wilcoxon test. Intra- and inter-observer agreement was analyzed using intraclass correlation coefficients (ICC). Lesion quantification by VNE and LGE were compared using linear regression and ICC. Results: 1348 HCM patients provided 4093 triplets of matched T1-maps, cines, and LGE datasets. After randomization and data quality control, 2695 datasets were used for VNE method development, and 345 for independent testing. VNE had significantly better image quality than LGE, as assessed by 4 operators (n=345 datasets, p<0.001, Wilcoxon test). VNE revealed characteristic HCM lesions in high visuospatial agreement with LGE. In 121 patients (n=326 datasets), VNE correlated with LGE in detecting and quantifying both hyper-intensity myocardial lesions (r=0.77-0.79, ICC=0.77-0.87; p<0.001) and intermediate-intensity lesions (r=0.70-0.76, ICC=0.82-0.85; p<0.001). The native CMR images (cine plus T1-map) required for VNE can be acquired within 15 minutes. Producing a VNE image takes less than one second. Conclusions: VNE is a new CMR technology that resembles conventional LGE, without the need for contrast administration. VNE achieved high agreement with LGE in the distribution and quantification of lesions, with significantly better image quality.


2021 ◽  
Vol 8 ◽  
Author(s):  
Cristian Izquierdo ◽  
Guillem Casas ◽  
Carlos Martin-Isla ◽  
Victor M. Campello ◽  
Andrea Guala ◽  
...  

Left Ventricular (LV) Non-compaction (LVNC), Hypertrophic Cardiomyopathy (HCM), and Dilated Cardiomyopathy (DCM) share morphological and functional traits that increase the diagnosis complexity. Additional clinical information, besides imaging data such as cardiovascular magnetic resonance (CMR), is usually required to reach a definitive diagnosis, including electrocardiography (ECG), family history, and genetics. Alternatively, indices of hypertrabeculation have been introduced, but they require tedious and time-consuming delineations of the trabeculae on the CMR images. In this paper, we propose a radiomics approach to automatically encode differences in the underlying shape, gray-scale and textural information in the myocardium and its trabeculae, which may enhance the capacity to differentiate between these overlapping conditions. A total of 118 subjects, including 35 patients with LVNC, 25 with HCM, 37 with DCM, as well as 21 healthy volunteers (NOR), underwent CMR imaging. A comprehensive radiomics characterization was applied to LV short-axis images to quantify shape, first-order, co-occurrence matrix, run-length matrix, and local binary patterns. Conventional CMR indices (LV volumes, mass, wall thickness, LV ejection fraction—LVEF—), as well as hypertrabeculation indices by Petersen and Jacquier, were also analyzed. State-of-the-art Machine Learning (ML) models (one-vs.-rest Support Vector Machine—SVM—, Logistic Regression—LR—, and Random Forest Classifier—RF—) were used for one-vs.-rest classification tasks. The use of radiomics models for the automated diagnosis of LVNC, HCM, and DCM resulted in excellent one-vs.-rest ROC-AUC values of 0.95 while generating these results without the need for the delineation of the trabeculae. First-order and texture features resulted to be among the most discriminative features in the obtained radiomics signatures, indicating their added value for quantifying relevant tissue patterns in cardiomyopathy differential diagnosis.


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