scholarly journals GW27-e0893 Clinical and MRI characteristics of end-stage hypertrophic cardiomyopathy

2016 ◽  
Vol 68 (16) ◽  
pp. C158-C159
Author(s):  
Cheng Sainan ◽  
Shihua Zhao
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
End Stage ◽  
Mri Characteristics

scholarly journals Right ventricular mobile thrombus in end-stage hypertrophic cardiomyopathy

2017 ◽  
Vol 15 (5) ◽  
pp. 173-175
Author(s):  
Takahiro Kusume ◽  
Shoichi Kubokawa ◽  
Noriyoshi Kaname ◽  
Yoko Nakaoka ◽  
Toshiaki Kotani ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Right Ventricular ◽  
End Stage

Pathology of End-stage Remodeling in a Family of Cats with Hypertrophic Cardiomyopathy

2005 ◽  
Vol 42 (4) ◽  
pp. 458-467 ◽  
Author(s):  
M. F. Cesta ◽  
C. J. Baty ◽  
B. W. Keene ◽  
I. W. Smoak ◽  
D. E. Malarkey
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Inflammatory Cell ◽  
Interventricular Septum ◽  
Coronary Vessels ◽  
Left Ventricular ◽  
Coronary Vessel ◽  
End Stage ◽  
Mononuclear Inflammatory Cell ◽  
Fractional Shortening

End-stage hypertrophic cardiomyopathy (ES-HCM), affecting 5-10% of human hypertrophic cardiomyopathy (HCM) patients, is characterized by relative thinning of the ventricular walls and septum with dilation of the ventricular lumen, decreased fractional shortening, and progression to heart failure. C. J. Baty and others recently documented similar progressive changes to ES-HCM in a family of four cats through serial echocardiograms. At the time of heart failure, these cats exhibited changes similar to those exhibited by human ES-HCM patients. Our objectives were to describe the pathologic alterations associated with ES-HCM and investigate the pathogenesis in three of the four cats. Grossly, there was left atrial dilation with relative thinning of the interventricular septum (IVS) and left ventricular free wall (LVFW). The left atrium contained large thrombi in two of the three cats, and all three cats died following thromboembolization of the aortic bifurcation. Histologically, all three cats had subendocardial and myocardial fibrosis, predominantly of the IVS and LVFW, and one cat had acute, multifocal, myocardial infarcts with mononuclear inflammatory cell infiltrates. The pathogenesis of ES-HCM is uncertain, but theories implicate occlusion of the coronary blood flow by thickening of the coronary vessels, coronary vascular thromboembolism or coronary vessel spasm, apoptosis of myocytes, and myocardial hypertrophy beyond the ability of the vasculature to supply blood. Apoptosis assays did not reveal any apoptotic myocytes. Considering the hypercoagulative state of these cats, coronary vascular thromboembolism could be a major contributing factor. We cannot exclude apoptosis or coronary vessel spasm on the basis of the data presented.

scholarly journals Prevalence, Clinical Profile, and Significance of Left Ventricular Remodeling in the End-Stage Phase of Hypertrophic Cardiomyopathy

Circulation ◽  
2006 ◽  
Vol 114 (3) ◽  
pp. 216-225 ◽  
Author(s):  
Kevin M. Harris ◽  
Paolo Spirito ◽  
Martin S. Maron ◽  
Andrey G. Zenovich ◽  
Francesco Formisano ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ventricular Remodeling ◽  
Left Ventricular Remodeling ◽  
Left Ventricular ◽  
Clinical Profile ◽  
End Stage

scholarly journals Single nucleus RNA-sequencing reveals altered intercellular communication and dendritic cell activation in nonobstructive hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Christina J Codden ◽  
Amy Larson ◽  
Junya Awata ◽  
Gayani Perera ◽  
Michael T Chin
Keyword(s):  
Gene Expression ◽  
Extracellular Matrix ◽  
Hypertrophic Cardiomyopathy ◽  
Growth Factor ◽  
Rna Sequencing ◽  
Intercellular Communication ◽  
Inhibitor Activity ◽  
Factor Binding ◽  
Single Nucleus ◽  
End Stage

End stage, nonobstructive hypertrophic cardiomyopathy (HCM) is an intractable condition with no disease-specific therapies. To gain insights into the pathogenesis of nonobstructive HCM, we performed single nucleus RNA-sequencing (snRNA-seq) on human HCM hearts explanted at the time of cardiac transplantation and organ donor hearts serving as controls. Differential gene expression analysis revealed 64 differentially expressed genes linked to specific cell types and molecular functions. Analysis of ligand-receptor pair gene expression to delineate potential intercellular communication revealed significant reductions in expressed ligand-receptor pairs affecting the extracellular matrix, growth factor binding, peptidase regulator activity, platelet-derived growth factor binding and protease binding in the HCM tissue. Changes in Integrin-beta1 receptor expression were responsible for many changes related to extracellular matrix interactions, by increasing in dendritic, smooth muscle and pericyte cells while decreasing in endothelial and fibroblast cells, suggesting potential mechanisms for fibrosis and microvascular disease in HCM and a potential role for dendritic cells. In contrast, there was an increase in ligand-receptor pair expression associated with adenylate cyclase binding, calcium channel molecular functions, channel inhibitor activity, ion channel inhibitor activity, phosphatase activator activity, protein kinase activator activity and titin binding, suggesting important shifts in various signaling cascades in nonobstructive, end stage HCM.

scholarly journals Heart transplantation in patients with hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Marta Farrero Torres ◽  
Felix Perez-Villa
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Life Expectancy ◽  
Sudden Death ◽  
Systolic Dysfunction ◽  
Treatment Options ◽  
Advanced Heart Failure ◽  
Clinical Presentations ◽  
End Stage ◽  
Ventricular Filling

[first paragraph of article]Hypertrophic cardiomyopathy has a broad spectrum of clinical presentations, from asymptomatic to patients with advanced heart failure and sudden death. Treatment options are limited, especially in non-obstructive forms. A minority of patients (around 3.5%) can progress to an end-stage state, characterized by systolic dysfunction and restrictive ventricular filling, related to extensive fibrotic replacement and chamber remodeling. In these cases, life expectancy is significantly reduced: a mean 3-year survival time has been reported.

A simple algorithm for a clinical step-by-step approach in the management of hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Georgios Efthimiadis ◽  
Thomas Zegkos ◽  
Despoina Parcharidou ◽  
Dimitris Ntelios ◽  
Theofilos Panagiotidis ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ventricular Hypertrophy ◽  
Therapeutic Strategy ◽  
Cardiopulmonary Exercise Testing ◽  
Simple Algorithm ◽  
Left Ventricular ◽  
Ambulatory Electrocardiography ◽  
Detailed Family History ◽  
End Stage ◽  
Sarcomeric Genes

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease with an autosomal dominant pattern and a reported prevalence of about 0.2%. In this review, we present a simple algorithm for the management of first diagnosed HCM patients. Initially, the clinical examination, medical and detailed family history and the ECG are essential. The etiological diagnosis of left ventricular hypertrophy is important in order to differentiate HCM due to sarcomeric genes mutation from other phenocopies, such as cardiac amyloidosis. The next step consists of the cardiovascular imaging and ambulatory electrocardiography. Cardiopulmonary exercise testing may also be considered if available. All of the above provide evidence for the critical step of the risk stratification of patients for sudden cardiac death. The therapeutic strategy, with respect to obstructive and nonobstructive disease, arrhythmias and end-stage HCM is also described.

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