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https://www.readbyqxmd.com/read/28630914/deregulated-ca-2-cycling-underlies-the-development-of-arrhythmia-and-heart-disease-due-to-mutant-obscurin
#1
Li-Yen R Hu, Maegen A Ackermann, Peter A Hecker, Benjamin L Prosser, Brendan King, Kelly A O'Connell, Alyssa Grogan, Logan C Meyer, Christopher E Berndsen, Nathan T Wright, W Jonathan Lederer, Aikaterini Kontrogianni-Konstantopoulos
Obscurins are cytoskeletal proteins with structural and regulatory roles encoded by OBSCN. Mutations in OBSCN are associated with the development of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). Specifically, the R4344Q mutation present in immunoglobulin domain 58 (Ig58) was the first to be linked with the development of HCM. To assess the effects of R4344Q in vivo, we generated the respective knock-in mouse model. Mutant obscurins are expressed and incorporated normally into sarcomeres...
June 2017: Science Advances
https://www.readbyqxmd.com/read/28629808/physiological-and-pathophysiological-role-of-transient-receptor-potential-canonical-channels-in-cardiac-myocytes
#2
REVIEW
Azmi A Ahmad, Molly Streiff, Chris Hunter, Qinghua Hu, Frank B Sachse
Transient receptor potential canonical (TRPC) channels constitute a family of seven Ca(2+) permeable ion channels, named TRPC1 to 7. These channels are abundantly expressed in the mammalian heart, yet mechanisms underlying activation of TRPC channels and their precise role in cardiac physiology remain poorly understood. In this review, we perused original literature regarding TRPC channels in cardiomyocytes. We first reviewed studies on TRPC channel assembly and sub-cellular localization across multiple species and cell types...
June 16, 2017: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/28624663/role-of-exercise-electrocardiogram-to-screen-for-t-wave-oversensing-after-implantation-of-subcutaneous-implantable-cardioverter-defibrillator
#3
Muhammad R Afzal, Christopher Evenson, Auroa Badin, Dilesh Patel, Hemant Godara, Michael Essandoh, Toshimasa Okabe, Jaret Tyler, Mahmoud Houmsse, Ralph Augostini, John Hummel, Steven Kalbfleisch, Emile G Daoud, Raul Weiss
BACKGROUND: During early experience with subcutaneous implantable cardioverter defibrillator (S-ICD(TM)), several patients had inappropriate shocks from TWOS during exercise. This prompted some operators to perform routine treadmill exercise test after implantation of S-ICD to screen for TWOS. Meanwhile, improvements have been made in the detection algorithms by the manufacturer. OBJECTIVE: To assess whether routine treadmill exercise post S-ICD(TM) implantation is warranted...
June 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28615213/experimental-and-human-evidence-for-lipocalin-2-neutrophil-gelatinase-associated-lipocalin-ngal-in-the-development-of-cardiac-hypertrophy-and-heart-failure
#4
Francine Z Marques, Priscilla R Prestes, Sean G Byars, Scott C Ritchie, Peter Würtz, Sheila K Patel, Scott A Booth, Indrajeetsinh Rana, Yosuke Minoda, Stuart P Berzins, Claire L Curl, James R Bell, Bryan Wai, Piyush M Srivastava, Antti J Kangas, Pasi Soininen, Saku Ruohonen, Mika Kähönen, Terho Lehtimäki, Emma Raitoharju, Aki Havulinna, Markus Perola, Olli Raitakari, Veikko Salomaa, Mika Ala-Korpela, Johannes Kettunen, Maree McGlynn, Jason Kelly, Mary E Wlodek, Paul A Lewandowski, Lea M Delbridge, Louise M Burrell, Michael Inouye, Stephen B Harrap, Fadi J Charchar
BACKGROUND: Cardiac hypertrophy increases the risk of developing heart failure and cardiovascular death. The neutrophil inflammatory protein, lipocalin-2 (LCN2/NGAL), is elevated in certain forms of cardiac hypertrophy and acute heart failure. However, a specific role for LCN2 in predisposition and etiology of hypertrophy and the relevant genetic determinants are unclear. Here, we defined the role of LCN2 in concentric cardiac hypertrophy in terms of pathophysiology, inflammatory expression networks, and genomic determinants...
June 14, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28614966/persistently-elevated-nuchal-translucency-and-the-fetal-heart
#5
Trisha V Vigneswaran, Tessa Homfray, Lindsey D Allan, John M Simpson, Vita Zidere
We describe the outcome of fifteen cases with an elevated nuchal translucency (NT) which persisted into the second trimester as nuchal edema (NE) > 6 mm whom underwent fetal echocardiography. Cases were identified following retrospective review of cardiac and genetic findings with NE. Minor congenital heart disease was identified in 3/15 by the second trimester. Agenesis of the ductus venosus was evident in four. Pulmonary valve stenosis was diagnosed in one fetus at the 20-week scan and hypertrophic cardiomyopathy in one...
June 14, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28613082/impact-of-the-implantable-cardioverter-defibrillator-on-confidence-to-undertake-physical-activity-in-inherited-heart-disease-a-cross-sectional-study
#6
Joanna Sweeting, Kylie Ball, Julie McGaughran, John Atherton, Christopher Semsarian, Jodie Ingles
BACKGROUND: Physical activity is associated with improved quality of life. Patients with an implantable cardioverter defibrillator (ICD) face unique clinical and psychological challenges. Factors such as fear of ICD shock may negatively impact on physical activity, while a sense of protection gained from the ICD may instil confidence to be active. AIM: We aimed to examine the impact of an ICD on physical activity levels and factors associated with amount of activity...
June 1, 2017: European Journal of Cardiovascular Nursing
https://www.readbyqxmd.com/read/28612913/the-usefulness-of-soluble-st2-and-galectin-3-as-novel-biomarkers-for-better-risk-stratification-in-hypertrophic-cardiomyopathy
#7
Monika Gawor, Mateusz Śpiewak, Jadwiga Janas, Katarzyna Kożuch, Aleksandra Wróbel, Łukasz Mazurkiewicz, Rafał Baranowski, Magdalena Marczak, Jacek Grzybowski
BACKGROUND: Estimation of sudden cardiac death (SCD) risk is an integral part of clinical management of patients with hypertrophic cardiomyopathy (HCM). Identification of novel biomarkers of this disease can provide additional criteria for SCD risk stratification. Soluble suppression of tumorigenicity (sST2) and galectin-3 (Gal-3) are useful biomarkers for prognosis of heart failure (HF). Both of them appear to mediate cardiac fibrosis - an important pathogenetic process in HCM. Data about sST2 and Gal-3 usefulness in patients with HCM are limited...
June 14, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28611684/protective-role-for-lpa3-in-cardiac-hypertrophy-induced-by-myocardial-infarction-but-not-by-isoproterenol
#8
Lin Cai, Guangpu Fan, Fang Wang, Si Liu, Tiewei Li, Xiangfeng Cong, Jerold Chun, Xi Chen
Background: We previously reported that lysophosphatidic acid (LPA) promoted cardiomyocyte hypertrophy in vitro via one of its G protein-coupled receptor subtypes, LPA3. In this study, we examined the role of LPA3 in cardiac hypertrophy induced by isoproterenol (ISO) and myocardial infarction. Methods:In vitro, neonatal rat cardiomyocytes (NRCMs) were subjected to LPA3 knocked-down, or pretreated with a β-adrenergic receptor (β-AR) antagonist (propranolol) before LPA/ISO treatment. Cardiomyocyte size and hypertrophic gene (ANP, BNP) mRNA levels were determined...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28611091/inhibition-of-microrna-146a-and-overexpression-of-its-target-dihydrolipoyl-succinyltransferase-protect-against-pressure-overload-induced-cardiac-hypertrophy-and-dysfunction
#9
Ward A Heggermont, Anna-Pia Papageorgiou, Annelies Quaegebeur, Sophie Deckx, Paolo Carai, Wouter Verhesen, Guy Eelen, Sandra Schoors, Rick van Leeuwen, Sergey Alekseev, Ies Elzenaar, Stefan Vinckier, Peter Pokreisz, Ann-Sophie Walravens, Rik Gijsbers, Chris Van Den Haute, Alexander G Nickel, Blanche Schroen, Marc van Bilsen, Stefan Janssens, Christoph Maack, Yigal M Pinto, Peter Carmeliet, Stephane Heymans
Background -Cardiovascular diseases remain the predominant cause of death worldwide, with the prevalence of heart failure continuing to increase. Despite increased knowledge of the metabolic alterations that occur in heart failure, novel therapies to treat the observed metabolic disturbances are still lacking. Methods -Mice were subjected to pressure overload by means of angiotensin-II infusion or transversal aortic constriction (TAC). MicroRNA-146a was either genetically or pharmacologically knocked out, or genetically overexpressed in cardiomyocytes...
June 13, 2017: Circulation
https://www.readbyqxmd.com/read/28610577/coronary-angiogenic-effect-of-long-term-administration-of-nigella-sativa
#10
Lubna I Al Asoom
BACKGROUND: Coronary angiogenesis is one of the preferable adaptive responses of aerobic training. Previous studies found inotropic and hypertrophic cardiac effects for long-term administration of Nigella sativa (NS), but no studies have explored its coronary angiogenic effect. The present study compared the effect of long-term NS- administration and exercise training on the induction of coronary angiogenesis. METHOD: Fifteen adult male Wistar rats were divided into three groups: control, NS-fed, and exercise-trained (Ex)...
June 13, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28600096/alcohol-septal-ablation-to-reduce-heart-failure
#11
REVIEW
Joshua McKay, Sherif F Nagueh
Hypertrophic cardiomyopathy is a commonly encountered inheritable cardiac disorder with variable phenotypic expression. Although most patients will have no or mild symptoms, 10% will develop heart failure symptoms refractory to medical management. This article discusses the mechanisms through which hypertrophic cardiomyopathy induces heart failure and how alcohol septal ablation can reverse each of these mechanisms to lead to clinical improvement.
July 2017: Interventional cardiology clinics
https://www.readbyqxmd.com/read/28600085/invasive-hemodynamics-of-myocardial-disease-systolic-and-diastolic-dysfunction-and-hypertrophic-obstructive-cardiomyopathy
#12
REVIEW
Michael Eskander, Morton J Kern
Heart failure is a clinical diagnosis that is supported by various laboratory, imaging, and invasive hemodynamic measures. There is no single diagnostic test. A variety of structural and/or functional myocardial abnormalities can lead to the inability of the heart to fill or eject blood. Despite ejection fraction being the most commonly assessed measure of systolic function in clinical practice, it is a poor measure of contractility because it is susceptible to loading conditions and chamber size. Invasive hemodynamic assessment remains of great importance in the evaluation of patients with myocardial disease or hypertrophic cardiomyopathy...
July 2017: Interventional cardiology clinics
https://www.readbyqxmd.com/read/28597791/value-of-latent-outflow-obstruction-to-predict-clinical-course-of-patients-with-hypertrophic-cardiomyopathy
#13
Fatih Bayrak, Gokhan Kahveci, Ali Buturak, Tansu Karaahmet, Ulaankhuu Batgerel, Atilla Koyuncu, Bulent Mutlu
Objective The frequency, significance and prognostic value of left ventricle obstruction (LVO) induced with provocation (latent LVO) is controversial for hypertrophic cardiomyopathy (HC) patients. This study was designed to assess the value of latent LVO in predicting the clinical course in 101 patients with HC. Methods and results Patients were followed for a mean of 82 ± 48 months (range 2 to 148 months) for clinical end points defined as a composite of cardiovascular death resuscitated cardiac arrest, appropriate defibrillator shock or hospitalization due to worsening of heart failure symptoms...
April 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28596175/calcium-signaling-and-cardiac-arrhythmias
#14
REVIEW
Andrew P Landstrom, Dobromir Dobrev, Xander H T Wehrens
There has been a significant progress in our understanding of the molecular mechanisms by which calcium (Ca(2+)) ions mediate various types of cardiac arrhythmias. A growing list of inherited gene defects can cause potentially lethal cardiac arrhythmia syndromes, including catecholaminergic polymorphic ventricular tachycardia, congenital long QT syndrome, and hypertrophic cardiomyopathy. In addition, acquired deficits of multiple Ca(2+)-handling proteins can contribute to the pathogenesis of arrhythmias in patients with various types of heart disease...
June 9, 2017: Circulation Research
https://www.readbyqxmd.com/read/28595881/outcomes-of-alcohol-septal-ablation-in%C3%A2-younger%C3%A2-patients-with-obstructive-hypertrophic%C3%A2-cardiomyopathy
#15
Max Liebregts, Lothar Faber, Morten K Jensen, Pieter A Vriesendorp, Jaroslav Januska, Jan Krejci, Peter R Hansen, Hubert Seggewiss, Dieter Horstkotte, Radka Adlova, Henning Bundgaard, Jurriën M Ten Berg, Josef Veselka
OBJECTIVES: The aim of this study was to describe the safety and outcomes of alcohol septal ablation (ASA) in younger patients with obstructive hypertrophic cardiomyopathy. BACKGROUND: The American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for older patients and patients with serious comorbidities. Data on long-term age-specific outcomes after ASA are scarce. METHODS: A total of 1,197 patients (mean age 58 ± 14 years) underwent ASA for obstructive hypertrophic cardiomyopathy...
June 12, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28594148/analysis-of-selected-genes-associated-with-cardiomyopathy-by%C3%A2-next-generation-sequencing
#16
Viktoria Szabadosova, Iveta Boronova, Peter Ferenc, Iveta Tothova, Jarmila Bernasovska, Michaela Zigova, Jan Kmec, Ivan Bernasovsky
BACKGROUND: As the leading cause of congestive heart failure, cardiomyopathy represents a heterogenous group of heart muscle disorders. Despite considerable progress being made in the genetic diagnosis of cardiomyopathy by detection of the mutations in the most prevalent cardiomyopathy genes, the cause remains unsolved in many patients. High-throughput mutation screening in the disease genes for cardiomyopathy is now possible because of using target enrichment followed by next-generation sequencing...
June 8, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28593149/effects-of-a-proteasome-inhibitor-on-cardiomyocytes-in-a-pressure-overload-hypertrophy-rat-model-an-animal-study
#17
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/28587741/plasma-level-of-big-endothelin-1-predicts-the-prognosis-in-patients-with-hypertrophic-cardiomyopathy
#18
Yilu Wang, Yida Tang, Yubao Zou, Dong Wang, Ling Zhu, Tao Tian, Jizheng Wang, Jingru Bao, Rutai Hui, Lianming Kang, Lei Song, Ji Wang
BACKGROUND: Cardiac remodeling is one of major pathological process in hypertrophic cardiomyopathy (HCM). Endothelin-1 has been linked to cardiac remodeling. Big endothelin-1 is the precursor of endothelin-1. METHODS: A total of 245 patients with HCM were enrolled from 1999 to 2011 and partitioned to low, middle and high level groups according to their plasma big endothelin-1 levels. RESULTS: At baseline, significant associations were found between high level of big endothelin-1 and left atrium size, heart function and atrial fibrillation...
April 20, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28582432/in-vivo-efficacy-of-the-akt-inhibitor-arq-092-in-noonan-syndrome-with-multiple-lentigines-associated-hypertrophic-cardiomyopathy
#19
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/28580208/heart-transplantation-in-patients-with-dystrophinopathic-cardiomyopathy-review-of-the-literature-and-personal-series
#20
REVIEW
Andrea Antonio Papa, Paola D'Ambrosio, Roberta Petillo, Alberto Palladino, Luisa Politano
Cardiomyopathy associated with dystrophinopathies [Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy (XL-dCM) and cardiomyopathy of Duchenne/Becker (DMD/BMD) carriers] is an increasing recognized manifestation of these neuromuscular disorders and notably contributes to their morbidity and mortality. Dystrophinopathic cardiomyopathy (DCM) is the result of the dystrophin protein deficiency at the myocardium level, parallel to the deficiency occurring at the skeletal muscle level...
May 2017: Intractable & Rare Diseases Research
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