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Hypertrophy cardiomyopathy

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https://www.readbyqxmd.com/read/28646025/remodeling-of-repolarization-and-arrhythmia-susceptibility-in-a-myosin-binding-protein-c-knockout-mouse-model
#1
Amir Toib, Chen Zhang, Giulia Borghetti, Xiaoxiao Zhang, Markus Wallner, Yijun Yang, Constantine Troupes, Hajime Kubo, Thomas Sharp, Eric Feldsott, Remus M Berretta, Neil Zalavadia, Danielle Trappanese, Shavonn Harper, Polina Gross, Xiongwen Chen, Sadia Mohsin, Steven Houser
Hypertrophic cardiomyopathy (HCM) is one of the most common genetic cardiac diseases and amongst the leading causes of sudden cardiac death (SCD) in the young. The cellular mechanisms leading to SCD in HCM are not well known. Prolongation of the action potential duration (APD) is a common feature predisposing hypertrophied hearts to SCD. Previous studies have explored the roles of inward Na(+) and Ca(2+) in the development of HCM, but the role of repolarizing K(+) currents have not been defined. The objective of this study was to characterize the arrhythmogenic phenotype and cellular electrophysiological properties of mice with HCM, induced by Myosin Binding Protein C (MyBPC) Knockout (KO) and to test the hypothesis that remodeling of repolarizing K(+) currents cause APD prolongation in MyBPC KO myocytes...
June 23, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28640453/corrigendum
#2
(no author information available yet)
Diabetic cardiomyopathy (DCM) was first recognized more than four decades ago and occurred independent of cardiovascular diseases or hypertension in both type 1 and type 2 diabetic patients. The exact mechanisms underlying this disease remain incompletely understood. Several pathophysiological bases responsible for DCM have been proposed, including the presence of hyperglycemia, nonenzymatic glycosylation of large molecules (e.g., proteins), energy metabolic disturbance, mitochondrial damage and dysfunction, impaired calcium handling, reactive oxygen species formation, inflammation, cardiac cell death, and cardiac hypertrophy and fibrosis, leading to impairment of cardiac contractile functions...
June 18, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28639774/-discovery-of-left-ventricular-hypertrophy-during-adult-echocardiography
#3
Nicolas B Dayal, Hajo Müller
Left ventricular hypertrophy is a common finding during echocardiography. A precise evaluation of the left ventricular wall thickness, ventricular mass and distribution of hypertrophy is crucial both for diagnostic workup, follow-up and for prognostic evaluation. The differential diagnosis of left ventricular hypertrophy includes hypertrophic cardiomyopathies, hypertrophy secondary to abnormal left ventricular filling conditions, hypertrophy linked to intense physical training and the isolated basal septal hypertrophy of the elderly...
May 24, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28639223/imaging-of-left-ventricular-hypertrophy-a-practical-utility-for-differential-diagnosis-and-assessment-of-disease-severity
#4
REVIEW
Toru Kubo, Hiroaki Kitaoka
PURPOSE OF REVIEW: Left ventricular hypertrophy (LVH) is often encountered in clinical practice, and it is a risk factor for cardiac mortality and morbidity. Determination of the etiology and disease severity is important for the management of patients with LVH. The aim of this review is to show the remarkable progress in cardiac imaging and its importance in clinical practice. RECENT FINDINGS: This review focuses on clinical features and characteristic cardiac imaging in LVH caused by various diseases including hypertension, aortic valve stenosis, hypertrophic cardiomyopathy, and secondary cardiomyopathies...
August 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28638577/a-rare-case-of-apical-hypertrophic-cardiomyopathy-ahcm
#5
Seyed Abbas Mirabbasi, Koroush Khalighi, Suresh Mukkamala, Archana Kodali
Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy that involves thickening of the distal portion of the left ventricular wall. Most commonly seen in the Japan, with a prevalence rate of about 15% of all HCM patient, its incidence in the USA is approximately 3% of HCM cases. We report a case of a 46-year-old woman with history of hypertension who presented to emergency department with worsening dyspnea and orthopnea with features of left ventricular hypertrophy (LVH) and diffuse large T-wave inversions in the lateral leads on a 12-lead ECG...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28624653/giant-ring-mitochondria-in-a-patient-with-heart-failure-and-cerebral-white-matter-disease-due-to-mt-tl1-mitochondrial-gene-mutation
#6
Brian A Houston, Daniel P Judge, Emily Brown, Marc Halushka, Lili A Barouch
BACKGROUND: The presence of giant ring mitochondria on endomyocardial biopsy is rarely reported and does not have a well-defined differential diagnosis. METHODS: We report the case of a 54-year-old man with heart failure with preserved ejection fraction and left ventricular hypertrophy, initially thought to have an infiltrative cardiomyopathy. RESULTS: The patient was found to have extensive vacuolization caused by the presence of giant ring mitochondria on endomyocardial biopsy...
June 14, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28624223/evaluation-of-mybpc3-trans-splicing-and-gene-replacement-as-therapeutic-options-in-human-ipsc-derived-cardiomyocytes
#7
Maksymilian Prondzynski, Elisabeth Krämer, Sandra D Laufer, Aya Shibamiya, Ole Pless, Frederik Flenner, Oliver J Müller, Julia Münch, Charles Redwood, Arne Hansen, Monica Patten, Thomas Eschenhagen, Giulia Mearini, Lucie Carrier
Gene therapy is a promising option for severe forms of genetic diseases. We previously provided evidence for the feasibility of trans-splicing, exon skipping, and gene replacement in a mouse model of hypertrophic cardiomyopathy (HCM) carrying a mutation in MYBPC3, encoding cardiac myosin-binding protein C (cMyBP-C). Here we used human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from an HCM patient carrying a heterozygous c.1358-1359insC MYBPC3 mutation and from a healthy donor. HCM hiPSC-CMs exhibited ∼50% lower MYBPC3 mRNA and cMyBP-C protein levels than control, no truncated cMyBP-C, larger cell size, and altered gene expression, thus reproducing human HCM features...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28617969/conditionally-targeted-deletion-of-psen1-leads-to-diastolic-heart-dysfunction
#8
Xiao-Wei Song, Qing-Ning Yuan, Ying Tang, Mi Cao, Ya-Feng Shen, Zhen-Yu Zeng, Chang-Hai Lei, SongHua Li, Xian-Xian Zhao, Yong-Ji Yang
Recently, PSEN1 has been reported to have mutations in dilated cardiomyopathy pedigrees. However, the function and mechanism of PSEN1 in cardiomyopathy remains unresolved. Here, we established 4 types of genetically modified mice to determine the function of PSEN1 in cardiac development and pathology. PSEN1 null mutation resulted in perinatal death, retardation of heart growth, ventricular dilatation, septum defects, and valvular thickening. PSEN1 knockout in adults led to decreased muscle fibers, widened sarcomere Z lines and reduced lengths of sarcomeres in cardiomyocytes...
June 15, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28612277/3-0%C3%A2-t-magnetic-resonance-myocardial-perfusion-imaging-for-semi-quantitative-evaluation-of-coronary-microvascular-dysfunction-in-hypertrophic-cardiomyopathy
#9
Liang Yin, Hai-Yan Xu, Sui-Sheng Zheng, Ying Zhu, Jiang-Xi Xiao, Wei Zhou, Si-Si Yu, Liang-Geng Gong
This study aimed to assess coronary microvascular dysfunction (CMD) differences in hypertrophic cardiomyopathy (HCM) patients using cardiac magnetic resonance (CMR) first-pass perfusion and late gadolinium enhancement imaging. Forty-seven patients with HCM and twenty-one healthy volunteers underwent CMR at rest. Imaging protocols included short axis cine, first-pass myocardial perfusion, and late gadolinium enhancement (LGE). Left ventricular end-diastolic wall thickness (EDTH), LGE, time to peak (Tpeak), maximal up-slope (Slopemax), and peak signal intensity (SIpeak) were assessed for each myocardial segment...
June 13, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28611128/decreased-kcne2-expression-participates-in-the-development-of-cardiac-hypertrophy-by-regulation-of-calcineurin-nfat-nuclear-factor-of-activated-t-cells-and-mitogen-activated-protein-kinase-pathways
#10
Wenjuan Liu, Jianxin Deng, Wenwen Ding, Gang Wang, Yuanyuan Shen, Junmeng Zheng, Xiaoming Zhang, Yizhi Luo, Chifei Lv, Yonghui Wang, Liqing Chen, Dewen Yan, Ryan L Boudreau, Long-Sheng Song, Jie Liu
BACKGROUND: KCNE2 is a promiscuous auxiliary subunit of voltage-gated cation channels. A recent work demonstrated that KCNE2 regulates L-type Ca(2+) channels. Given the important roles of altered Ca(2+) signaling in structural and functional remodeling in diseased hearts, this study investigated whether KCNE2 participates in the development of pathological hypertrophy. METHODS AND RESULTS: We found that cardiac KCNE2 expression was significantly decreased in phenylephrine-induced cardiomyocyte hypertrophy in neonatal rat ventricular myocytes and in transverse aortic constriction-induced cardiac hypertrophy in mice, as well as in dilated cardiomyopathy in human...
June 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28607615/diffuse-interstitial-fibrosis-assessed-by-cardiac-magnetic-resonance-is-associated-with-dispersion-of-ventricular-repolarization-in-patients-with-hypertrophic-cardiomyopathy
#11
David Hurtado-de-Mendoza, Celia P Corona-Villalobos, Iraklis Pozios, Jorge Gonzales, Yalda Soleimanifard, Sanjay Sivalokanathan, Diego Montoya-Cerrillo, Styliani Vakrou, Ihab Kamel, Wilfredo Mormontoy-Laurel, Ketty Dolores-Cerna, Jacsel Suarez, Sergio Perez-Melo, David A Bluemke, Theodore P Abraham, Stefan L Zimmerman, M Roselle Abraham
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is characterized by myocyte hypertrophy, disarray, fibrosis, and increased risk for ventricular arrhythmias. Increased QT dispersion has been reported in patients with HCM, but the underlying mechanisms have not been completely elucidated. In this study, we examined the relationship between diffuse interstitial fibrosis, replacement fibrosis, QTc dispersion and ventricular arrhythmias in patients with HCM. We hypothesized that fibrosis would slow impulse propagation and increase dispersion of ventricular repolarization, resulting in increased QTc dispersion on surface electrocardiogram (ECG) and ventricular arrhythmias...
June 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/28593726/the-evolution-of-basal-septal-hypertrophy-from-benign-and-age-related-normal-variant-to-potentially-obstructive-and-symptomatic-cardiomyopathy
#12
REVIEW
Anthony C Pearson
Localized thickening of the basal portion of the ventricular septum or basal septal hypertrophy (BSH) has been identified both at autopsy and by imaging studies for decades; despite numerous investigations, there is no consensus on the significance of this finding and a remarkable lack of consistency in terminology. This paper summarizes the scientific literature on the topic, focusing on recent echocardiographic findings. A case description illustrating some of the complex issues involved in measurement and diagnosis and differentiation from sigmoidal hypertrophic cardiomyopathy (HCM) is presented...
June 8, 2017: Echocardiography
https://www.readbyqxmd.com/read/28593149/effects-of-a-proteasome-inhibitor-on-cardiomyocytes-in-a-pressure-overload-hypertrophy-rat-model-an-animal-study
#13
In-Sub Kim, Won-Min Jo
BACKGROUND: The ubiquitin-proteasome system (UPS) is an important pathway of proteolysis in pathologic hypertrophic cardiomyocytes. We hypothesize that MG132, a proteasome inhibitor, might prevent hypertrophic cardiomyopathy (CMP) by blocking the UPS. Nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) and androgen receptor (AR) have been reported to be mediators of CMP and heart failure. This study drew upon pathophysiologic studies and the analysis of NF-κB and AR to assess the cardioprotective effects of MG132 in a left ventricular hypertrophy (LVH) rat model...
June 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28588076/pai-1-controls-cardiomyocyte-tgf-%C3%AE-and-cardiac-fibrosis
#14
Panagiotis Flevaris, Sadiya S Khan, Mesut Eren, Adam J Schuldt, Sanjiv J Shah, Daniel C Lee, Sweta Gupta, Amy Shapiro, Paul Burridge, Asish K Ghosh, Douglas E Vaughan
Background -Fibrosis is the pathologic consequence of stress-induced tissue remodeling and matrix accumulation. Increased levels of plasminogen activator inhibitor type I (PAI-1) have been shown to promote fibrosis in multiple organ systems. Paradoxically, homozygous genetic deficiency of PAI-1 is associated with spontaneous age-dependent cardiac-selective fibrosis in mice. We have identified a novel PAI-1-dependent mechanism that regulates cardiomyocyte-derived fibrogenic signals and cardiac transcriptional pathways during injury...
June 6, 2017: Circulation
https://www.readbyqxmd.com/read/28586098/diastolic-dysfunction-of-hypertrophic-cardiomyopathy-genotype-positive-subjects-without-hypertrophy-is-detected-by-tissue-doppler-imaging-a-systematic-review-and-meta-analysis
#15
Wen Liu, Dandan Sun, Jun Yang
OBJECTIVES: To evaluate whether diastolic dysfunction derived by tissue Doppler imaging (TDI) would be an earlier manifestation in genotype-positive hypertrophic cardiomyopathy (HCM) subjects without left ventricular hypertrophy (LVH). METHODS: We systematically searched Pubmed, Medline, and Web of Science with an upper date limit of June 2016 for studies evaluating the diastolic function of HCM genotype-positive subjects without hypertrophy (G+/LVH-). Based on the inclusion criteria, eligible studies were selected...
June 6, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28582432/in-vivo-efficacy-of-the-akt-inhibitor-arq-092-in-noonan-syndrome-with-multiple-lentigines-associated-hypertrophic-cardiomyopathy
#16
Jianxun Wang, Vasanth Chandrasekhar, Giovanni Abbadessa, Yi Yu, Brian Schwartz, Maria I Kontaridis
Noonan Syndrome with Multiple Lentigines (NSML, formerly LEOPARD syndrome) is an autosomal dominant "RASopathy" disorder manifesting in congenital heart disease. Most cases of NSML are caused by catalytically inactivating mutations in the protein tyrosine phosphatase (PTP), non-receptor type 11 (PTPN11), encoding the SH2 domain-containing PTP-2 (SHP2) protein. We previously generated knock-in mice harboring the PTPN11 mutation Y279C, one of the most common NSML alleles; these now-termed SHP2Y279C/+ mice recapitulate the human disorder and develop hypertrophic cardiomyopathy (HCM) by 12 weeks of age...
2017: PloS One
https://www.readbyqxmd.com/read/28573739/apical-hypertrophic-cardiomyopathy-with-left-ventricular-apical-aneurysm-importance-of-multi-modality-imaging
#17
Nisharahmed Kherada, Juan M Vinardell, Christos G Mihos, Orlando Santana
Apical hypertrophic cardiomyopathy (HCM) is an uncommon variant of HCM characterized by apical hypertrophy without the septal predominance seen in the majority of HCM cases. In 2% of patients, a concomitant left ventricular apical aneurysm is observed, which increases the risk of sudden death and adverse HCM-related events. Multimodality imaging is helpful for appropriate identification of this particular morphologic pattern. Herein, we present a case of apical HCM with a left ventricular apical aneurysm, exemplifying the utility of a multimodality approach from resting electrocardiogram, transthoracic echocardiogram, left ventriculography, and cardiac magnetic resonance imaging, for proper risk stratification and treatment planning...
June 1, 2017: Echocardiography
https://www.readbyqxmd.com/read/28565941/insulin-replacement-limits-progression-of-diabetic-cardiomyopathy-in-the-low-dose-streptozotocin-induced-diabetic-rat
#18
Mitchel Tate, Minh Deo, Anh H Cao, Sally G Hood, Karina Huynh, Helen Kiriazis, Xiao-Jun Du, Tracey L Julius, Gemma A Figtree, Gregory J Dusting, David M Kaye, Rebecca H Ritchie
Diabetic cardiomyopathy is a major contributor to the increasing burden of heart failure globally. Effective therapies remain elusive, in part due to the incomplete understanding of the mechanisms underlying diabetes-induced myocardial injury. The objective of this study was to assess the direct impact of insulin replacement on left ventricle structure and function in a rat model of diabetes. Male Sprague-Dawley rats were administered streptozotocin (55 mg/kg i.v.) or citrate vehicle and were followed for 8 weeks...
May 1, 2017: Diabetes & Vascular Disease Research
https://www.readbyqxmd.com/read/28553897/diagnostic-score-for-the-detection-of-cardiac-amyloidosis-in-patients-with-left-ventricular-hypertrophy-and-impact-on-prognosis
#19
Eve Cariou, Youssef Bennani Smires, Gérard Victor, Guillaume Robin, David Ribes, Pierre Pascal, Antoine Petermann, Pauline Fournier, Stanislas Faguer, Jérôme Roncalli, Hervé Rousseau, Dominique Chauveau, Didier Carrié, Isabelle Berry, Michel Galinier, Olivier Lairez
BACKGROUND: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH. METHODS AND RESULTS: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a (99m)Tc-hydroxymethylene-diphosphonate scintigraphy ((99m)Tc-HMDP) allowing to discriminate three groups of diagnoses: CA (n = 50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n = 19) and unspecific cardiomyopathy (n = 45)...
May 29, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28552325/hypertrophic-cardiomyopathy-and-left-ventricular-hypertrophy-in-hypertensive-heart-disease-with-mildly-reduced-or-preserved-ejection-fraction-insight-from-altered-mechanics-and-native-t1-mapping
#20
L-M Wu, D-A L An, Q-Y Yao, Y-R Z Ou, Q Lu, M Jiang, J-R Xu
AIM: To explore the relationship between extracellular volume (ECV), native T1, and systolic strain in hypertrophic cardiomyopathy (HCM) and hypertensive patients with left ventricular hypertrophy (HTN LVH) with mildly reduced or preserved ejection fraction. MATERIALS AND METHODS: T1 mapping was performed in 45 patients with late gadolinium enhancement positive (LGE+) HCM (mean age, 53±6 years), 11 patients with LGE- (LGE-) HCM (mean age, 56±5 years), and 20 patients with HTN LVH (mean age, 55±6 years) on at 3 T magnetic resonance imaging (MRI) using the modified look-locker inversion-recovery (MOLLI) pulse sequence...
May 25, 2017: Clinical Radiology
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