Fibrosis in hypertrophic cardiomyopathy: role of novel echo techniques and multi-modality imaging assessment

AbstractHypertrophic cardiomyopathy (HCM) represents one of the primary cardiomyopathies and may lead to heart failure and sudden cardiac death. Among various histologic features of the disease examined, assessment of myocardial fibrosis may offer valuable information, since it may be considered the common nominator for all HCM connected complications. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) has emerged as the reference noninvasive method for visualizing and quantifying myocardial fibrosis in patients with HCM. T1 mapping, a promising new CMR technique, may provide an advantage over conventional LGE-CMR, by permitting a more valid quantification of diffuse fibrosis. On the other hand, echocardiography offers a significantly more portable, affordable, and easily accessible solution for the study of fibrosis. Various echocardiographic techniques ranging from integrated backscatter and contrast-enhanced ultrasound to two- (2D) or three-dimensional (3D) deformation and shear wave imaging may offer new insights into substrate characterization in HCM. The aim of this review is to describe thoroughly all different modalities that may be used in everyday clinical practice for HCM fibrosis evaluation (with special focus on echocardiographic techniques), to concisely present available evidence and to argue in favor of multi-modality imaging application. It is essential to understand that the role of various imaging modalities is not competitive but complementary, ...
Source: Heart Failure Reviews - Category: Cardiology Source Type: research

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CONCLUSION: High performance was obtained by using DPS-Net in LV detection and LVEF measurement for heart failure with several phenotypes. High performance was observed in a large-scale dataset, suggesting that the DPS-Net was highly adaptive across different echocardiographic systems.PMID:34170197 | DOI:10.1152/ajpheart.00416.2020
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Source Type: research
CONCLUSIONS: Novel TNNT2-rs397516484, MYH6-rs372446459, and MYBPC3-rs786204339 are pathogenic sarcomere gene mutations in familial HCM, leading to decreased cardiac function and metabolic disturbances of carbohydrate metabolism, which have important implications for biologically defined diagnoses and precision medicine.PMID:34087240 | DOI:10.1016/j.cca.2021.05.034
Source: International Journal of Clinical Chemistry - Category: Chemistry Authors: Source Type: research
Am J Physiol Heart Circ Physiol. 2021 Apr 30. doi: 10.1152/ajpheart.00873.2020. Online ahead of print.ABSTRACTHeart failure presents as the leading cause of infant mortality in individuals with Barth syndrome (BTHS), a rare genetic disorder due to mutations in the tafazzin (TAZ) gene affecting mitochondrial structure and function. Investigations into the perturbed bioenergetics in the BTHS heart remain limited. Hence, our objective was to identify the potential alterations in myocardial energy metabolism and molecular underpinnings that may contribute to the early cardiomyopathy and heart failure development in BTHS. Cardi...
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Source Type: research
ConclusionThis article presents a summary of key clinical cardiology trials during the past year and should be of relevance to both clinicians and cardiology researchers.
Source: Advances in Therapy - Category: Drugs & Pharmacology Source Type: research
AbstractCurrent echocardiography techniques have allowed more precise assessment of cardiac structure and function of the several types of cardiomyopathies. Parameters derived from echocardiographic tissue imaging (ETI) —tissue Doppler, strain, strain rate, and others—are extensively used to provide a framework in the evaluation and management of cardiomyopathies. Generally, myocardial function assessed by ETI is depressed in all types of cardiomyopathies, non-ischemic dilated cardiomyopathy (DCM) in particular . In hypertrophic cardiomyopathy (HCM), ETI is useful to identify subclinical disease in family membe...
Source: Heart Failure Reviews - Category: Cardiology Source Type: research
Background— The noninvasive assessment of altered myocardium in patients with genetic mutations that are associated with hypertrophic cardiomyopathy (HCM) remains challenging. In this pilot study, we evaluated whether a novel echocardiography-based assessment of myocardial microstructure, the signal intensity coefficient (SIC), could detect tissue-level alterations in HCM sarcomere mutation carriers with and without left ventricular hypertrophy. Methods and Results— We studied 3 groups of genotyped individuals: sarcomere mutation carriers with left ventricular hypertrophy (clinical HCM; n=36), mutation carrier...
Source: Circulation: Heart Failure - Category: Cardiology Authors: Tags: Clinical Studies, Cardiomyopathy, Echocardiography, Imaging, Ultrasound Forum for Early Career Clinical Investigation Source Type: research
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