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Advances in Multi-Modality Imaging in Hypertrophic Cardiomyopathy.

ABSTRACT: Hypertrophic cardiomyopathy (HCM) is characterized by abnormal growth of the myocardium with myofilament disarray and myocardial hyper-contractility, leading to left ventricular hypertrophy and fibrosis. Where culprit genes are identified, they typically relate to cardiomyocyte sarcomere structure and function. Multi-modality imaging plays a crucial role in the diagnosis, monitoring, and risk stratification of HCM, as well as in screening those at risk. Following the recent publication of the first European Society of Cardiology (ESC) cardiomyopathy guidelines, we build on previous reviews and explore the roles of electrocardiography, echocardiography, cardiac magnetic resonance (CMR), cardiac computed tomography (CT), and nuclear imaging. We examine each modality's strengths along with their limitations in turn, and discuss how they can be used in isolation, or in combination, to facilitate a personalized approach to patient care, as well as providing key information and robust safety and efficacy evidence within new areas of research.

SUBMITTER: Goldie FC

PROVIDER: S-EPMC10856479 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature

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Advances in Multi-Modality Imaging in Hypertrophic Cardiomyopathy.

Goldie Fraser C FC Lee Matthew M Y MMY Coats Caroline J CJ Nordin Sabrina S

Journal of clinical medicine 20240201 3

Hypertrophic cardiomyopathy (HCM) is characterized by abnormal growth of the myocardium with myofilament disarray and myocardial hyper-contractility, leading to left ventricular hypertrophy and fibrosis. Where culprit genes are identified, they typically relate to cardiomyocyte sarcomere structure and function. Multi-modality imaging plays a crucial role in the diagnosis, monitoring, and risk stratification of HCM, as well as in screening those at risk. Following the recent publication of the fi ...[more]

PMID: 38337535

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Project description:Heart failure (HF) is recognized as a leading cause of morbidity and mortality worldwide. Dilated cardiomyopathy (DCM) is a common phenotype in patients presenting with HF. Timely diagnosis, appropriate identification of the underlying cause, individualized risk stratification, and prediction of clinical response to treatment have improved the prognosis of DCM over the last few decades. In this article, we reviewed the current evidence on available imaging techniques used for DCM patients. In this direction, we evaluated appropriate scenarios for the implementation of echocardiography, nuclear imaging, and cardiac computed tomography, and we focused on the primordial role that cardiac magnetic resonance (CMR) holds in the diagnosis, prognosis, and tailoring of therapeutic options in this population of special clinical interest. We explored the predictive value of CMR toward left ventricular reverse remodeling and prediction of sudden cardiac death, thus guiding the decisions for device therapy. Principles underpinning the use of state-of-the-art CMR techniques such as parametric mapping and feature-tracking strain analysis are also provided, along with expectations for the anticipated future advances in this field. We also attempted to correlate the evidence with clinical practice, with the intent to address questions on selecting the optimal imaging method for different indications and clinical needs. Overall, we recommend a comprehensive assessment of DCM patients at baseline and at follow-up intervals depending on the clinical status, with the addition of CMR as a second-line modality to other imaging techniques. We also provide an algorithm to guide the detailed imaging approach of the patient with DCM. We expect that future guidelines will upgrade their clinical recommendations for the utilization of CMR in DCM, which is expected to further improve the quality of care and the outcomes. This review provides an up-to-date perspective on the imaging of dilated cardiomyopathy patients and will be of clinical value to training doctors and physicians involved in the area of heart failure.

Project description:Introduction: Patients with hypertrophic cardiomyopathy (HCM) are at risk for lethal ventricular arrhythmia, but the electrophysiological substrate behind this is not well-understood. We used non-invasive electrocardiographic imaging to characterize patients with HCM, including cardiac arrest survivors. Methods: HCM patients surviving ventricular fibrillation or hemodynamically unstable ventricular tachycardia (n = 17) were compared to HCM patients without a personal history of potentially lethal arrhythmia (n = 20) and a pooled control group with structurally normal hearts. Subjects underwent exercise testing by non-invasive electrocardiographic imaging to estimate epicardial electrophysiology. Results: Visual inspection of reconstructed epicardial HCM maps revealed isolated patches of late activation time (AT), prolonged activation-recovery intervals (ARIs), as well as reversal of apico-basal trends in T-wave inversion and ARI compared to controls (p < 0.005 for all). AT and ARI were compared between groups. The pooled HCM group had longer mean AT (60.1 ms vs. 52.2 ms, p < 0.001), activation dispersion (55.2 ms vs. 48.6 ms, p = 0.026), and mean ARI (227 ms vs. 217 ms, p = 0.016) than structurally normal heart controls. HCM ventricular arrhythmia survivors could be differentiated from HCM patients without a personal history of life-threatening arrhythmia by longer mean AT (63.2 ms vs. 57.4 ms, p = 0.007), steeper activation gradients (0.45 ms/mm vs. 0.36 ms/mm, p = 0.011), and longer mean ARI (234.0 ms vs. 221.4 ms, p = 0.026). A logistic regression model including whole heart mean activation time and activation recovery interval could identify ventricular arrhythmia survivors from the HCM cohort, producing a C statistic of 0.76 (95% confidence interval 0.72-0.81), with an optimal sensitivity of 78.6% and a specificity of 79.8%. Discussion: The HCM epicardial electrotype is characterized by delayed, dispersed conduction and prolonged, dispersed activation-recovery intervals. Combination of electrophysiologic measures with logistic regression can improve differentiation over single variables. Future studies could test such models prospectively for risk stratification of sudden death due to HCM.

Project description:IntroductionMutations in the sarcomere protein, particularly in cardiac myosin binding protein C gene (MyBPC3), were the most frequent genetic cause of hypertrophic cardiomyopathy (HCM). The pathogenic MyBPC3 P459fs mutation has been reported in HCM patients. However, there was limited knowledge of the structure-function relationships and potential pathways in clinical HCM with MyBPC3 P459fs mutation.MethodsWe used multi-omics approaches and super-resolution imaging to explore the effects of MyBPC3 P459fs mutation on humans and cells. HCM patients carrying MyBPC3 P459fs mutation (MyBPC3-P459fs HCMs) and healthy controls (HCs) were evaluated for myocardial function using both conventional and advanced echocardiography. In parallel, H9C2 myocardial cells infected with either MyBPC3 P459fs mutation (P459fs cells) or its wild type (WT cells) were investigated for myocardial fiber formation and the potential pathways behind this using super-resolution imaging and metabolomics and proteomics.ResultsFirst, conventional and advanced echocardiography showed that MyBPC3-P459fs HCMs exhibited left ventricular diastolic and systolic dysfunction. Subsequently, super-resolution imaging indicated that P459fs cells formed fewer and shorter myocardial fibers in the cytoplasm compared to WT cells. Moreover, our metabolomic and proteomic data suggested several key components of mitochondrial membrane integrity, myocardial remodeling, myocardial energy metabolism, oxidative stress, inflammation, and actin binding capacity were significantly altered in response to P459fs mutation.ConclusionsThis investigation indicated myocardial dysfunction and myocardial fiber disarray in clinical HCMs with MyBPC3 P459fs mutation and added potential pathways underlying this. These findings provided a link between the observed structural and functional disorders in MyBPC3 P459fs mutation and its onset of HCM pathogenesis and might have a significant translational contribution to effective treatment in HCM patients with MyBPC3 P459fs mutation.

Dataset Information

Advances in Multi-Modality Imaging in Hypertrophic Cardiomyopathy.

Publications

Advances in Multi-Modality Imaging in Hypertrophic Cardiomyopathy.

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