Cardiovascular Magnetic Resonance Imaging of Myocardial Interstitial Expansion in Hypertrophic Cardiomyopathy.
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SUBMITTER: Wong TC
PROVIDER: S-EPMC3973947 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, affecting 1 out of 500 adults globally. It is a widely heterogeneous disorder characterized by a range of phenotypic expressions, and is most often identified by non-invasive imaging that includes echocardiography and cardiovascular magnetic resonance imaging (CMR). Within the last two decades, cardiac magnetic resonance imaging (MRI) has emerged as the defining tool for the characterization and prognostication of cardiomyopathies. With a higher image quality, spatial resolution, and the identification of morphological variants of HCM, CMR has become the gold standard imaging modality in the assessment of HCM. Moreover, it has been crucial in its management, as well as adding prognostic information that clinical history nor other imaging modalities may not provide. This literature review addresses the role and current applications of CMR, its capacity in evaluating HCM, and its limitations.
| S-EPMC8871211 | biostudies-literature
Project description:PurposeHCM is the most common inherited cardiomyopathy. Historically, there has been poor correlation between genotype and phenotype. However, CMR has the potential to more accurately assess disease phenotype. We characterized phenotype with CMR in a cohort of patients with confirmed HCM and high prevalence of genetic testing.MethodsPatients with a diagnosis of HCM, who had undergone contrast-enhanced CMR were identified. Left ventricular mass index (LVMI) and volumes were measured from steady-state free precession sequences. Late gadolinium enhancement (LGE) was quantified using the full width, half maximum method. All patients were prospectively followed for the development of septal reduction therapy, arrhythmia or death.ResultsWe included 273 patients, mean age 51.2 ± 15.5, 62.9% male. Of those patients 202 (74.0%) underwent genetic testing with 90 pathogenic, likely pathogenic, or rare variants and 13 variants of uncertain significance identified. Median follow-up was 1138 days. Mean LVMI was 82.7 ± 30.6 and 145 patients had late gadolinium enhancement (LGE). Patients with beta-myosin heavy chain (MYH7) mutations had higher LV ejection fraction (68.8 vs 59.1, p<0.001) than those with cardiac myosin binding protein C (MYBPC3) mutations. Patients with MYBPC3 mutations were more likely to have LVEF < 55% (29.7% vs 4.9%, p = 0.005) or receive a defibrillator than those with MYH7 mutations (54.1% vs 26.8%, p = 0.020).ConclusionsWe found that patients with MYBPC3 mutations were more likely to have impaired ventricular function and may be more prone to arrhythmic events. Larger studies using CMR phenotyping may be capable of identifying additional characteristics associated with less frequent genetic causes of HCM.
| S-EPMC6568393 | biostudies-literature
Project description:Dilated cardiomyopathy (DCM) is a common cause of non-ischaemic heart failure, conferring high morbidity and mortality, including sudden cardiac death due to systolic dysfunction or arrhythmic sudden death. Within the DCM cohort exists a group of patients with familial disease. In this article we review the pathophysiology and cardiac imaging findings of familial DCM, with specific attention to known disease subtypes. The role of advanced cardiac imaging cardiovascular magnetic resonance is still accumulating, and there remains much to be elucidated. We discuss its potential clinical roles as currently known, with respect to diagnostic utility and risk stratification. Advances in such risk stratification may help target pharmacological and device therapies to those at highest risk.
| S-EPMC10057087 | biostudies-literature
Project description:BackgroundTo elucidate the value of texture analysis (TA) in detecting and differentiating myocardial tissue alterations on T2-weighted CMR (cardiovascular magnetic resonance imaging) in patients with cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM).MethodsIn this retrospective study, 100 CA (58.5 ± 10.7 years; 41 (41%) females) and 217 HCM (50.7 ± 14.8 years, 101 (46.5%) females) patients who underwent CMR scans were included. Regions of interest for TA were delineated by two radiologists independently on T2-weighted imaging (T2WI). Stepwise dimension reduction and texture feature selection based on reproducibility, machine learning algorithms, and correlation analyses were performed to select features. Both the CA and HCM groups were randomly divided into a training dataset and a testing dataset (7:3). After the TA model was established in the training set, the diagnostic performance of the model was validated in the testing set and further validated in a subgroup of patients with similar hypertrophy.ResultsThe 7 independent texture features provided, in combination, a diagnostic accuracy of 86.0% (AUC = 0.915; 95% CI 0.879-0.951) in the training dataset and 79.2% (AUC = 0.842; 95% CI 0.759-0.924) in the testing dataset. The differential diagnostic accuracy in the similar hypertrophy subgroup was 82.2% (AUC = 0.864, 95% CI 0.805-0.922). The significance of the difference between the AUCs of the TA model and late gadolinium enhancement (LGE) was verified by Delong's test (p = 0.898). All seven texture features showed significant differences between CA and HCM (all p < 0.001).ConclusionsOur study demonstrated that texture analysis based on T2-weighted images could feasibly differentiate CA from HCM, even in patients with similar hypertrophy. The selected final texture features could achieve a comparable diagnostic capacity to the quantification of LGE. Trial registration Since this study is a retrospective observational study and no intervention had been involved, trial registration is waived.
| S-EPMC9124433 | biostudies-literature
Project description:AimsGenetic testing in relatives of hypertrophic cardiomyopathy (HCM) patients leads to early identification of pathogenic DNA variant carriers (G+), before the onset of left ventricular hypertrophy. Routine phenotyping consists of electrocardiography (ECG) and transthoracic echocardiography (TTE). Cardiovascular magnetic resonance (CMR) has become valuable in the work-up of HCM. In this study, we investigated the value of CMR in phenotyping of G+ family members.Methods and resultsThis study included 91 G+ subjects who underwent ECG, TTE and CMR, with a maximal wall thickness (MWT) <15 mm on TTE. The relative performance of TTE and CMR regarding wall thickness measurements and HCM diagnoses was assessed. HCM was defined as MWT of ≥13 mm. Logistic regression was performed to assess whether ECG and TTE parameters can predict CMR results. Most subjects (75%) had an MWT <13 mm on TTE, of which 23 (34%) were diagnosed with HCM based on CMR. MWT differences (range 1-10 mm) were often caused by an anterobasal hook-shaped thickening of the myocardium not visible on TTE. Two of 23 (9%) subjects with HCM on TTE were reclassified as no HCM on CMR. Normal ECG and TTE results almost excluded reclassifications by CMR. The prevalence of other HCM-related abnormalities on CMR was low.ConclusionCMR reclassified 27% of subjects. Subjects with normal ECG/TTE results were reclassified in a low number of cases, justifying screening with ECG and TTE in G+ relatives. In subjects with abnormal ECGs and/or poor TTE image quality, CMR is indicated.
| S-EPMC9365305 | biostudies-literature
Project description:Cardiovascular magnetic resonance (CMR) imaging has had a vast impact on the understanding of a wide range of disease processes and pathophysiological mechanisms. More recently, it has contributed significantly to the diagnosis and risk stratification of patients with valvular heart disease. With its increasing use, CMR allows for a detailed, reproducible, qualitative, and quantitative evaluation of left ventricular volumes and mass, thereby enabling assessment of the haemodynamic impact of a valvular lesion upon the myocardium. Postprocessing of the routinely acquired images with feature tracking CMR methodology can give invaluable information about myocardial deformation and strain parameters that suggest subclinical ventricular impairment that remains undetected by conventional measures such as the ejection fraction (EF). T1 mapping and late gadolinium enhancement (LGE) imaging provide deep myocardial tissue characterisation that is changing the approach towards risk stratification of patients as an increasing body of evidence suggests that the presence of fibrosis is related to adverse events and prognosis. This review summarises the current evidence regarding the utility of CMR in the left ventricular assessment of patients with aortic stenosis or mitral regurgitation and its value in diagnosis, risk stratification, and management.
| S-EPMC8888109 | biostudies-literature
Project description:BackgroundClinical data on myocardial function in HCM mutation carriers (carriers) is sparse but suggests that subtle functional abnormalities can be measured with tissue Doppler imaging before the development of overt hypertrophy. We aimed to confirm the presence of functional abnormalities using cardiovascular magnetic resonance (CMR), and to investigate if sensitive functional assessment could be employed to identify carriers.Results28 carriers and 28 controls were studied. Global left atrial (LA) and left ventricular (LV) dimensions, segmental peak systolic circumferential strain (SCS) and peak diastolic circumferential strain rate (DCSR), as well as the presence of late Gadolinium enhancement (LGE) were determined with CMR. Septal and lateral myocardial velocities were measured with echocardiographic tissue Doppler imaging. LV mass and volumes were comparable between groups. Maximal septal to lateral wall thickness ratio (SL ratio) was larger in carriers than in controls (1.3+/-0.2 versus 1.1+/-0.1, p<0.001). Also, LA volumes were larger in carriers compared to controls (p<0.05). Both peak SCS (p<0.05) and peak DCSR (p<0.01) were lower in carriers compared to controls, particularly in the basal lateral wall. Focal LGE was present in 2 carriers and not in controls. The combination of a SL ratio>1.2 and a peak DCSR<105%.s-1 was present in 45% of carriers and in none of the controls, yielding a positive predictive value of 100%. Two carriers and 18 controls had a SL ratio<1.2 and peak DCSR>105%.s-1, yielding a negative predictive value of 90%. With multivariate analysis, HCM mutation carriership was an independent determinant of reduced peak SCS and peak DCSR.ConclusionsHCM mutation carriership is an independent determinant of reduced peak SCS and peak DCSR when LV wall thickness is within normal limits, and is associated with increased LA volumes and SL ratio. Using SL ratio and peak DCSR has a high accuracy to identify carriers. However, since carriers also display structural abnormalities and focal LGE, we advocate to also evaluate morphology and presence of LGE when screening for carriers.
| S-EPMC2842263 | biostudies-literature
Project description: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.
| S-EPMC8586199 | biostudies-literature
Project description:PurposeTo investigate the incidence, mechanism, and risk factors of aortic regurgitation (AR) in patients with hypertrophic cardiomyopathy (HCM) by using echocardiography and cardiac magnetic resonance (CMR).Methods105 HCM patients, 52 hypertension (HTN) patients and 50 healthy controls (HC) were retrospectively recruited. HCM patients were divided into 38 with AR (HCMAR) subject and 67 without AR. The subaortic complex, D1 (the largest distance of the interventricular septum that protruded into the LVOT) and D3 (the LVOT effective width) were assessed and compared between the two groups of HCM patients.ResultsAR was more common in HCM than in HTN and HC (36 %, 17 %, and 10 %, respectively, P = 0.001). HCM patients with AR were older (58 ± 11 vs. 45 ± 16 years, P < 0.001) and had a higher incidence of hypertension (55 % vs. 33 %, P = 0.03). D1 was greater (13.5 ± 4.4 vs. 10.6 ± 4.0 mm, P = 0.001), and D3 was shorter in the HCMAR group (10.2 ± 5.3 vs. 13.7 ± 5.9 mm, P = 0.003). Anterior mitral leaflet length and left atrial diameter were greater in HCMAR group (all P < 0.05). On multivariable logistic regression analysis, the independent risk factors of AR in HCM patients were LVOTO and age.ConclusionsThis study demonstrated that AR is a common comorbidity of HCM, especially in patients with LVOTO. LVOTO and age were independent risk factors of AR in HCM patient.
| S-EPMC10822682 | biostudies-literature
Project description:BackgroundAngina is common in hypertrophic cardiomyopathy (HCM) and is associated with abnormal myocardial perfusion. Wave intensity analysis improves the understanding of the mechanics of myocardial ischemia.ObjectivesWave intensity analysis was used to describe the mechanisms underlying perfusion abnormalities in patients with HCM.MethodsSimultaneous pressure and flow were measured in the proximal left anterior descending artery in 33 patients with HCM and 20 control patients at rest and during hyperemia, allowing calculation of wave intensity. Patients also underwent quantitative first-pass perfusion cardiac magnetic resonance to measure myocardial perfusion reserve.ResultsPatients with HCM had a lower coronary flow reserve than control subjects (1.9 ± 0.8 vs. 2.7 ± 0.9; p = 0.01). Coronary hemodynamics in HCM were characterized by a very large backward compression wave during systole (38 ± 11% vs. 21 ± 6%; p < 0.001) and a proportionately smaller backward expansion wave (27% ± 8% vs. 33 ± 6%; p = 0.006) compared with control subjects. Patients with severe left ventricular outflow tract obstruction had a bisferiens pressure waveform resulting in an additional proximally originating deceleration wave during systole. The proportion of waves acting to accelerate coronary flow increased with hyperemia, and the magnitude of change was proportional to the myocardial perfusion reserve (rho = 0.53; p < 0.01).ConclusionsCoronary flow in patients with HCM is deranged. Distally, compressive deformation of intramyocardial blood vessels during systole results in an abnormally large backward compression wave, whereas proximally, severe left ventricular outflow tract obstruction is associated with an additional deceleration wave. Perfusion abnormalities in HCM are not simply a consequence of supply/demand mismatch or remodeling of the intramyocardial blood vessels; they represent a dynamic interaction with the mechanics of myocardial ischemia that may be amenable to treatment.
| S-EPMC5054113 | biostudies-literature