20,438 results match your criteria Cardiomyopathy Hypertrophic


[Diagnostic utility of creatine kinase elevation in the routine biochemical profile in cats].

Schweiz Arch Tierheilkd 2020 Jul;162(7):451-462

Abteilung für Kardiologie, Klinik für Kleintiermedizin, Vetsuisse-Fakultät, Universität Zürich.

Introduction: Creatine kinase (CK) is a muscle enzyme that is very sensitive to muscle damage. Therefore, serum CK is measured particularly to confirm suspected myopathy. Since 2013, this enzyme has been included in the routine chemistry profile in our hospital. Read More

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http://dx.doi.org/10.17236/sat00266DOI Listing

Dilated Cardiomyopathy Mutations in Thin Filament Regulatory Proteins Reduce Contractility, Suppress Systolic Ca & Activate NFAT & AKT Signalling.

Am J Physiol Heart Circ Physiol 2020 Jul 3. Epub 2020 Jul 3.

Radcliffe Department of Medicine (Division of Cardiovascular Medicine), University of Oxford, United Kingdom.

Dilated cardiomyopathy (DCM) is clinically characterised by dilated ventricular cavities and reduced ejection fraction, leading to heart failure and increased thromboembolic risk. Mutations in thin filament regulatory proteins can cause DCM and have been shown in vitro to reduce contractility and myofilament Ca-affinity. In this work we have studied the functional consequences of mutations in cardiac troponin T (R131W), cardiac troponin I (K36Q) and α-tropomyosin (E40K) using adenovirally transduced isolated guinea pig left ventricular cardiomyocytes. Read More

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http://dx.doi.org/10.1152/ajpheart.00272.2020DOI Listing

Atrial pacing and administration of nifekalant hydrochloride for unstable atrial fibrillation: a case report.

Eur Heart J Case Rep 2020 Jun 14;4(3):1-5. Epub 2020 May 14.

Department of Cardiology, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1, Jigyohama, Chuo-ku, Fukuoka, Fukuoka 810-8563, Japan.

Background: Atrial fibrillation (AF) is a common arrhythmia in patients with hypertrophic cardiomyopathy (HCM) and can deteriorate haemodynamic status.

Case Summary: We report a case of a 77-year-old woman with cardiogenic shock due to paroxysmal AF, complicated with HCM and aortic stenosis. Atrial fibrillation was successfully managed with temporary atrial pacing and administration of nifekalant hydrochloride without invasive mechanical circulatory support until surgery. Read More

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http://dx.doi.org/10.1093/ehjcr/ytaa093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319856PMC

Transcatheter closure of complex iatrogenic ventricular septal defect: a case report.

Eur Heart J Case Rep 2020 Jun 1;4(3):1-5. Epub 2020 Jun 1.

Department of Cardiac, Thoracic and Vascular Sciences, Cardiology Unit, University of Padua Medical School, Via Giustiniani, 2, 35128 Padua, Italy.

Background: Iatrogenic membranous ventricular septal defects (VSDs) are rare complications of cardiothoracic surgery, such as septal myectomy for hypertrophic obstructive cardiomyopathy (HOCM). Transcatheter closure is considered an appealing alternative to surgery, given the increased mortality associated with repeated surgical procedures, but reports are extremely limited.

Case Summary: We herein report the case of a 63-year-old woman with HOCM who underwent successful percutaneous closure of an iatrogenic VSD after septal myectomy. Read More

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http://dx.doi.org/10.1093/ehjcr/ytaa106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319813PMC

Rare association of left ventricular non-compaction and hypertrophic cardiomyopathy.

Eur Heart J Case Rep 2020 Jun 28;4(3):1-2. Epub 2020 May 28.

Heart Institute (InCor)/University of Sao Paulo Medical School, Clinical Unity of Cardiomyopathies, Av. Dr Eneas de Carvalho Aguiar, 44, CEP, 05403 000 Sao Paulo, Brazil.

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http://dx.doi.org/10.1093/ehjcr/ytaa109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319806PMC

Left Bundle Pacing for Left Bundle Branch Block and Intermittent Third-Degree Atrioventricular Block in a Mutation-Related Hypertrophic Cardiomyopathy With Restrictive Phenotype in a Child.

Front Pediatr 2020 16;8:312. Epub 2020 Jun 16.

Department of Cardiology, Children's Hospital of Nanjing Medical University, Nanjing, China.

Hypertrophic cardiomyopathy (HCM) is a group of myocardial diseases defined by cardiac hypertrophy which cannot be explained by secondary causes with a non-dilated left ventricle and preserved or increased ejection fraction. Sometimes it can be combined with restrictive cardiomyopathy. Here we describe a very rare case of a 12-year-old girl with non-obstructive hypertrophic cardiomyopathy accompanied by restrictive phenotype, complete left bundle branch block and intermittent third-degree atrioventricular block, who presented with recurrent syncope. Read More

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http://dx.doi.org/10.3389/fped.2020.00312DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7308432PMC

Loss of miR-132/212 Has No Long-Term Beneficial Effect on Cardiac Function After Permanent Coronary Occlusion in Mice.

Front Physiol 2020 16;11:590. Epub 2020 Jun 16.

Department of Cardiology, Experimental Cardiology Laboratory, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, Netherlands.

Myocardial infarction (MI) is caused by occlusion of the coronary artery and induces ischemia in the myocardium and eventually a massive loss in cardiomyocytes. Studies have shown many factors or treatments that can affect the healing and remodeling of the heart upon infarction, leading to better cardiac performance and clinical outcome. Previously, miR-132/212 has been shown to play an important role in arteriogenesis in a mouse model of hindlimb ischemia and in the regulation of cardiac contractility in hypertrophic cardiomyopathy in mice. Read More

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http://dx.doi.org/10.3389/fphys.2020.00590DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7309700PMC

Gender Disparities in Clinical Outcome After Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy in the Chinese Han Population: A Cohort Study.

Heart Lung Circ 2020 Jun 11. Epub 2020 Jun 11.

Department of Cardiology, The People's Hospital of China Medical University, The People's Hospital of Liaoning Province, Shenyang, Liaoning Province, P. R. China. Electronic address:

Background: Sex differences in the long-term prognosis of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients undergoing alcohol septal ablation (ASA) remain unclear, especially in the Chinese Han population.

Method: This cohort study included 320 HOCM Chinese Han patients who underwent ASA because of symptomatic left ventricular outflow tract (LVOT) obstruction. Patients were grouped according to sex: females (mean±standard deviation age [SD] 50. Read More

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http://dx.doi.org/10.1016/j.hlc.2020.04.014DOI Listing

Rotation and torsion of the left ventricle with cardiovascular magnetic resonance tagging: comparison of two analysis methods.

BMC Med Imaging 2020 Jul 1;20(1):73. Epub 2020 Jul 1.

HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, PO Box 340, FI-00029, Helsinki, HUS, Finland.

Background: Left ventricle rotation and torsion are fundamental components of myocardial function, and several software packages have been developed for analysis of these components. The purpose of this study was to compare the suitability of two software packages with different technical principles for analysis of rotation and torsion of the left ventricle during systole.

Methods: A group of hypertrophic cardiomyopathy (HCM) patients (N = 14, age 43 ± 11 years), mutation carriers without hypertrophy (N = 10, age 34 ± 13 years), and healthy relatives (N = 12, age 43 ± 17 years) underwent a cardiovascular magnetic resonance examination, including spatial modulation of magnetization tagging sequences in basal and apical planes of the left ventricle. Read More

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http://dx.doi.org/10.1186/s12880-020-00473-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7329530PMC

Outcomes and predictors of cardiac events in medically treated patients with atrial functional mitral regurgitation.

Int J Cardiol 2020 Jun 28. Epub 2020 Jun 28.

Department of Cardiovascular Medicine, Kobe City Medical Center General Hospital, Kobe, Japan.

Background: Little is known about the outcomes and predictors of adverse cardiac events in medically treated patients with atrial functional mitral regurgitation (FMR).

Methods: We screened 1405 consecutive patients with grade ≥ 3+ mitral regurgitation (MR) detected by echocardiography. After excluding patients with previous or early (within 3 months from diagnosis) mitral valve surgery, congenital heart disease, hypertrophic cardiomyopathy, severe aortic valve disease, or unknown etiology, the study population consisted of 319 patients with primary MR, 395 patients with FMR with left ventricular (LV) dysfunction, and 184 patients with atrial FMR. Read More

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http://dx.doi.org/10.1016/j.ijcard.2020.06.042DOI Listing

Diagnosis and Screening of Patients with Fabry Disease.

Ther Clin Risk Manag 2020 22;16:551-558. Epub 2020 Jun 22.

Department of Medicine I, Klinikum Vest, Knappschaftskrankenhaus Recklinghausen, Academic Teaching Hospital, Ruhr-University Bochum, Recklinghausen, Germany.

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by absence or deficient activity of α-galactosidase A (α-Gal A) due to mutations in the α-galactosidase A gene (), leading to progressive accumulation of globotriaosylceramide (Gb3) in tissues and organs including heart, kidney, the eyes, vascular endothelium, the nervous system and the skin. Cardiac involvement is leading to fatal complications and reduced life expectancy. FD is treatable with disease-specific treatment (enzyme replacement therapy (ERT) or with chaperone therapy). Read More

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http://dx.doi.org/10.2147/TCRM.S247814DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319521PMC

Isolated Heme A Synthase from Aquifex aeolicus Is a Trimer.

mBio 2020 Jun 30;11(3). Epub 2020 Jun 30.

Department of Molecular Membrane Biology, Max Planck Institute of Biophysics, Frankfurt am Main, Germany

The integral membrane protein heme A synthase (HAS) catalyzes the biosynthesis of heme A, which is a prerequisite for cellular respiration in a wide range of aerobic organisms. Previous studies have revealed that HAS can form homo-oligomeric complexes, and this oligomerization appears to be evolutionarily conserved among prokaryotes and eukaryotes and is shown to be essential for the biological function of eukaryotic HAS. Despite its importance, little is known about the detailed structural properties of HAS oligomers. Read More

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http://dx.doi.org/10.1128/mBio.02615-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7327177PMC

Alcohol septal ablation in obstructive hypertrophic cardiomyopathy is a safe procedure in experienced hands.

Rev Port Cardiol 2020 Jun 27. Epub 2020 Jun 27.

Universitätsklinikum Wuerzburg, Deutsches Zentrum für Herzinsuffizienz (DZHI), Comprehensive Heart Failure Center (CHFC), Wuerzburg, Germany.

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http://dx.doi.org/10.1016/j.repc.2020.06.003DOI Listing

Kappa-light Chain Amyloid Overlapping Hypertrophic Cardiomyopathy With Myocardial Noncompaction.

Circ Cardiovasc Imaging 2020 Jul 1;13(7):e010379. Epub 2020 Jul 1.

Department of Cardiovascular, Respiratory, Nephrologic, Anesthesiologic and Geriatric Sciences (A.F., M.A., C.C.), Sapienza University, Rome.

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http://dx.doi.org/10.1161/CIRCIMAGING.119.010379DOI Listing

BET bromodomain proteins regulate transcriptional reprogramming in genetic dilated cardiomyopathy.

JCI Insight 2020 Jun 30. Epub 2020 Jun 30.

Department of Medicine, Emory University School of Medicine, Atlanta, United States of America.

The bromodomain and extraterminal (BET) family of epigenetic reader proteins are key regulators of inflammatory and hypertrophic gene expression in the heart. We previously identified the activation of pro-inflammatory gene networks as a key early driver of dilated cardiomyopathy (DCM) in transgenic mice expressing a mutant form of phospholamban (PLNR9C) - a genetic cause of DCM in humans. We hypothesized that BETs coactivate this inflammatory process, representing a critical node in the progression of DCM. Read More

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http://dx.doi.org/10.1172/jci.insight.138687DOI Listing

Evolutionary dissection of mtDNA hg H: a susceptibility factor for hypertrophic cardiomyopathy.

Mitochondrial DNA A DNA Mapp Seq Anal 2020 Jun 30:1-7. Epub 2020 Jun 30.

Department for Congenital Disorders, Statens Serum Institute, Copenhagen, Denmark.

Mitochondrial DNA (mtDNA) haplogroup (hg) H has been reported as a susceptibility factor for hypertrophic cardiomyopathy (HCM). This was established in genetic association studies, however, the SNP or SNP's that are associated with the increased risk have not been identified. Hg H is the most frequent European mtDNA hg with greater than 80 subhaplogroups (subhgs) each defined by specific SNPs. Read More

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http://dx.doi.org/10.1080/24701394.2020.1782897DOI Listing

Assessment of left ventricular systolic function in hypertrophic cardiomyopathy patients with myocardial injury: a study based on layer-specific speckle tracking echocardiaography.

Int J Cardiovasc Imaging 2020 Jun 29. Epub 2020 Jun 29.

Department of Cardiovascular Ultrasound, The First Affiliated Hospital of China Medical University, 155 North Nanjing Street, Heping District, Shenyang, 110001, China.

We conducted this study to investigate left ventricle (LV) systolic function in endocardial, mid-myocardial, and epicardial layers by two-dimensional (2D) speckle tracking echocardiography (STE) in hypertrophic cardiomyopathy (HCM) patients with myocardial injury indexed by elevated serum cardiac troponin I (cTnI). Twenty-nine HCM patients with myocardial injury, thirty-five HCM patients without myocardial injury, and ninty-one healthy controls were enrolled in this study. Serum cTnI > 0. Read More

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http://dx.doi.org/10.1007/s10554-020-01921-6DOI Listing

Diastolic Vortex Alterations with Reducing Left Ventricular Volume: An in Vitro Study.

J Biomech Eng 2020 Jun 30. Epub 2020 Jun 30.

School of Mechanical and Aerospace Engineering, Oklahoma State University, 201 General Academic Building, Stillwater, OK 74078.

Despite the large number of studies of intraventricular filling dynamics for potential clinical applications, little is known as to how the diastolic vortex ring properties are altered with reduction in internal volume of the cardiac left ventricle (LV). The latter is of particular importance in LV diastolic dysfunction and in congenital diseases such as hypertrophic cardiomyopathy (HCM), where LV hypertrophy can reduce LV internal volume. We hypothesized that peak circulation and the rate of decay of circulation of the diastolic vortex would be altered with reducing end diastolic volume (EDV) due to increasing confinement. Read More

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http://dx.doi.org/10.1115/1.4047663DOI Listing

[Hypertrophic cardiomyopathy: modern aspects of pharmacologic treatment].

Ter Arkh 2019 Sep 15;91(9):129-136. Epub 2019 Sep 15.

Pavlov Medical University.

This article discusses recent advances in understanding genetic basis and classification of hypertrophic cardiomyopathy. Here, we review pharmacologic treatment strategies and new developments in disease - specific management of HCM. Read More

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http://dx.doi.org/10.26442/00403660.2019.09.000137DOI Listing
September 2019

[Characterisation of circulating microRNA-21 levels in patients with hypertrophic cardiomyopathy].

Ter Arkh 2020 May 19;92(4):51-56. Epub 2020 May 19.

Pavlov First Saint Petersburg State Medical University.

Aim: To study the levels of circulating microRNA-21 in patients with hypertrophic cardiomyopathy (HCM) of different ages.

Materials And Methods: The study included 49 patients with HCM. The proportion of females was 55. Read More

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http://dx.doi.org/10.26442/00403660.2020.04.000272DOI Listing

Difference in Metabolomic Response to Exercise between Patients with and without Hypertrophic Cardiomyopathy.

J Cardiovasc Transl Res 2020 Jun 27. Epub 2020 Jun 27.

Cardiology Division, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.

It is unclear how hypertrophic cardiomyopathy (HCM) affects cardiac metabolic pathways at rest and with exercise. This case-control study compared 15 cases with HCM to 2 control groups without HCM. Metabolomic profiling of 210 metabolites was carried out at rest and at peak exercise. Read More

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http://dx.doi.org/10.1007/s12265-020-10051-2DOI Listing

Prevalence of sudden arrhythmic death syndrome-related genetic mutations in an Asian cohort of whole genome sequence.

Europace 2020 Jun 28. Epub 2020 Jun 28.

Division of Cardiology, Department of Internal Medicine and Cardiovascular center, National Taiwan University College of Medicine and Hospital, No. 7, Chung-Shan South Road, Taipei 100, Taiwan.

Aims: Recently, the spectrum of background mutation in the genes implicated in sudden arrhythmic death syndrome (SADS), has been elucidated in the Caucasian populations. However, this information is largely unknown in the Asian populations.

Methods And Results: We assessed the background rare variants (minor allele frequency < 0. Read More

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http://dx.doi.org/10.1093/europace/euaa092DOI Listing

Advanced myocardial characterization in hypertrophic cardiomyopathy: feasibility of CMR-based feature tracking strain analysis in a case-control study.

Eur Radiol 2020 Jun 25. Epub 2020 Jun 25.

PET-CT/MR Department, Harbin Medical University Cancer Hospital, Harbin, China.

Objectives: This study aimed to evaluate the feasibility and reproducibility of using cardiovascular magnetic resonance feature tracking (CMR-FT) for analysis of bi-ventricular strain and strain rate (SR) in hypertrophic cardiomyopathy (HCM) patients as well as to explore the correlation between right ventricular (RV) and left ventricular (LV) deformation.

Methods: A total of 60 HCM patients and 48 controls were studied. Global and segmental peak values of bi-ventricular longitudinal, circumferential, radial strain, and systolic SR were analyzed. Read More

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http://dx.doi.org/10.1007/s00330-020-06922-6DOI Listing

Assessment of left atrial electro-mechanical delay to predict atrial fibrillation in hypertrophic cardiomyopathy.

Eur Heart J Cardiovasc Imaging 2020 Jun 26. Epub 2020 Jun 26.

Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2300 RC Leiden, The Netherlands.

Aims: Atrial fibrillation (AF) is frequently observed in hypertrophic cardiomyopathy (HCM) and is associated with poor clinical outcome. Total atrial conduction time, estimated by tissue Doppler imaging (TDI), the so-called PA-TDI duration, reflects the left atrial (LA) structural and electrical remodelling. The aim of this study was to evaluate the association between PA-TDI and new-onset AF in patients with HCM. Read More

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http://dx.doi.org/10.1093/ehjci/jeaa174DOI Listing

CRISPR-Cas9 generated Pompe knock-in murine model exhibits early-onset hypertrophic cardiomyopathy and skeletal muscle weakness.

Sci Rep 2020 Jun 25;10(1):10321. Epub 2020 Jun 25.

Department of Pediatrics, University of California-Irvine School of Medicine, Irvine, CA, 92697, USA.

Infantile-onset Pompe Disease (IOPD), caused by mutations in lysosomal acid alpha-glucosidase (Gaa), manifests rapidly progressive fatal cardiac and skeletal myopathy incompletely attenuated by synthetic GAA intravenous infusions. The currently available murine model does not fully simulate human IOPD, displaying skeletal myopathy with late-onset hypertrophic cardiomyopathy. Bearing a Cre-LoxP induced exonic disruption of the murine Gaa gene, this model is also not amenable to genome-editing based therapeutic approaches. Read More

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http://dx.doi.org/10.1038/s41598-020-65259-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316971PMC

The Role of Mitral Valve in Hypertrophic Obstructive Cardiomyopathy: An Updated Review.

Curr Probl Cardiol 2020 Jun 4:100641. Epub 2020 Jun 4.

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and the most common cause of sudden cardiac death in young population, especially in athletes under 35 years old. Mutations occur primarily in the β-myosin heavy chain gene and involve the cardiac myosin-binding protein C gene. In this review we would like to focus on the importance of the examination of mitral valve apparatus and the mitral valve abnormalities in patients with HCM. Read More

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http://dx.doi.org/10.1016/j.cpcardiol.2020.100641DOI Listing

Hypertrophic cardiomyopathy masquerading as sarcoidosis: cases illustrating cardiac imaging overlap relative to pathology.

Cardiovasc Pathol 2020 May 19;49:107234. Epub 2020 May 19.

Centre for Heart Lung Innovation, St Paul's Hospital/University of British Columbia, Vancouver, BC, Canada; Departments of Pathology and Laboratory Medicine, Providence Health Care/University of British Columbia, Vancouver, BC, Canada.

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http://dx.doi.org/10.1016/j.carpath.2020.107234DOI Listing

Tyrosyl phosphorylation of PZR promotes hypertrophic cardiomyopathy in PTPN11-associated Noonan syndrome with multiple lentigines.

JCI Insight 2020 Jun 25. Epub 2020 Jun 25.

Department of Pharmacology, Yale University School of Medicine, New Haven, United States of America.

Noonan syndrome with multiple lentigines (NSML) is a rare autosomal dominant disorder that presents with cardio-cutaneous-craniofacial defects. Hypertrophic cardiomyopathy (HCM) represents the major life-threatening presentation in NSML. Mutations in the PTPN11 gene that encodes for the protein tyrosine phosphatase (PTP), SHP2, represents the predominant cause of HCM in NSML. Read More

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http://dx.doi.org/10.1172/jci.insight.137753DOI Listing

Hypertrophic and Dilated Cardiomyopathy-Associated Troponin T Mutations R130C and ΔK210 Oppositely Affect Length-Dependent Calcium Sensitivity of Force Generation.

Front Physiol 2020 3;11:516. Epub 2020 Jun 3.

Institute of Vegetative Physiology, University of Cologne, Cologne, Germany.

Length-dependent activation of calcium-dependent myocardial force generation provides the basis for the Frank-Starling mechanism. To directly compare the effects of mutations associated with hypertrophic cardiomyopathy and dilated cardiomyopathy, the native troponin complex in skinned trabecular fibers of guinea pigs was exchanged with recombinant heterotrimeric, human, cardiac troponin complexes containing different human cardiac troponin T subunits (hcTnT): hypertrophic cardiomyopathy-associated hcTnT, dilated cardiomyopathy-associated hcTnT or the wild type hcTnT (hcTnT) serving as control. Force-calcium relations of exchanged fibers were explored at short fiber length defined as 110% of slack length () and long fiber length defined as 125% of (1. Read More

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http://dx.doi.org/10.3389/fphys.2020.00516DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7283609PMC

Pseudo-Paradoxical Jet Flow in a Patient with Midventricular Obstructive Hypertrophic Cardiomyopathy.

CASE (Phila) 2020 Jun 12;4(3):179-188. Epub 2020 Apr 12.

Department of Cardiovascular Medicine, Kumamoto University School of Medicine, Kumamoto, Japan.

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http://dx.doi.org/10.1016/j.case.2020.02.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303248PMC

Usefulness of ECG to differentiate apical hypertrophic cardiomyopathy from non-ST elevation acute coronary syndrome.

BMC Cardiovasc Disord 2020 Jun 23;20(1):306. Epub 2020 Jun 23.

Department of Cardiology, The Second Affiliated Hospital of Dalian Medical University, Dalian, Liaoning, China.

Background: Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of nonobstructive HCM. ApHCM is characterized by left ventricular hypertrophy involve the distal apex. The electrocardiographic character of ApHCM can mimic non-ST elevation acute coronary syndrome (NSTEACS), triggering a series of studies and treatments that may be unnecessary. Read More

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http://dx.doi.org/10.1186/s12872-020-01592-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310283PMC

Utilization and Complications of Catheter Ablation for Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy.

J Am Heart Assoc 2020 Jun 23:e015721. Epub 2020 Jun 23.

The Azrieli Faculty of Medicine in the Galilee Bar-Ilan University Safed Israel.

Background Atrial fibrillation (AF) is common and bears a major clinical impact in patients with hypertrophic cardiomyopathy (HCM). We aimed to investigate the use and real-world safety of catheter ablation for AF in patients with HCM. Methods and Results We drew data from the US National Inpatient Sample to identify cases of AF ablation in HCM patients between 2003 and 2015. Read More

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http://dx.doi.org/10.1161/JAHA.119.015721DOI Listing

From Other Journals: A Review of Recent Articles in Pediatric Cardiology.

Authors:
Tarek Alsaied

Pediatr Cardiol 2020 Jun 23. Epub 2020 Jun 23.

Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address the following: (1) transcatheter closure of sinus venous atrial septal defect which is feasible in adults with careful planning, (2) exercise echocardiography in children with hypertrophic cardiomyopathy which was found to be very helpful to identify low-risk patients when there is no gradient at rest or during exercise, (3) features of Fontan patients with normal exercise capacity who typically report more active life style and have less complications during the post-Fontan admissions, (4) the natural history of Anderson-Tawil syndrome which is characterized by high incidence of life-threatening arrhythmogenic events especially when there is history of syncope, ventricular tachycardia, or the use of amiodarone, (5) pulmonary artery banding in complete atrioventricular septal defects which were found to be a safe alternative to early complete repair and served as a bridge to biventricular repair with variable effect on common atrioventricular valve regurgitation and (6) tricuspid valve interventions (TVI) in patients undergoing pulmonary valve replacement (PVR) which further reduce tricuspid regurgitation beyond what is expected in PVR alone without increasing early complications or hospital length of stay. Read More

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http://dx.doi.org/10.1007/s00246-020-02404-0DOI Listing

Cardiac hypertrophic risk markers of left ventricle and left atrium in chronic heart failure due to aortic and mitral valve disease.

Acta Radiol 2020 Jun 22:284185120933530. Epub 2020 Jun 22.

Faculty of Biological Sciences, Department of Biochemistry, Quaid-I-Azam University, Islamabad, Pakistan.

Background: Chronic valvular heart disease leads to systolic dysfunction and left atrial enlargement that ultimately results in heart failure.

Purpose: To investigate prognostic importance of Echocardiography and plasma natriuretic peptide levels that increase as a compensatory response and can be used as predictive markers for cardiac hypertrophy.

Material And Methods: The patients were divided into three groups: 51 with left ventricle hypertrophy due to aortic valve disease; 126 with left atrial enlargement due to mitral valve dysfunction; and 76 with both conditions. Read More

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http://dx.doi.org/10.1177/0284185120933530DOI Listing

Septal Myectomy in patients with previous coronary revascularization - hypertrophic cardiomyopathy masquerading as ischemic heart disease.

Int J Cardiol 2020 Jun 20. Epub 2020 Jun 20.

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA.

Background: Patients with obstructive hypertrophic cardiomyopathy (HCM) may have symptoms mimicking ischemic heart disease, including chest pain and shortness of breath. Some patients undergo coronary revascularization which may not lead to symptomatic improvement. This study assesses clinical presentations and outcomes of patients with previous coronary revascularization undergoing septal myectomy. Read More

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http://dx.doi.org/10.1016/j.ijcard.2020.06.015DOI Listing

Silencing of MYH7 ameliorates disease phenotypes in human iPSC-Cardiomyocytes.

Physiol Genomics 2020 Jun 22. Epub 2020 Jun 22.

Cardiovascular Medicine, Stanford University, United States.

Allele-specific RNA silencing has been shown to be an effective therapeutic treatment in a number of diseases, including neurodegenerative disorders. Studies of allele-specific silencing in hypertrophic cardiomyopathy (HCM) to date have focused on mouse models of disease. We here examine allele-specific silencing in a human-cell model of HCM. Read More

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http://dx.doi.org/10.1152/physiolgenomics.00021.2020DOI Listing

Stillbirth in women with diabetes: a retrospective analysis of fetal autopsy reports.

J Matern Fetal Neonatal Med 2020 Jun 21:1-8. Epub 2020 Jun 21.

Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, University of Rochester Medical Center, Rochester, NY, USA.

Diabetes in pregnancy is associated with an increased rate of stillbirth. There are a wide variety of factors that have been implicated including placental insufficiency, hypoxia, hyperinsulinemia and impaired cardiac function. Furthermore, there is evidence that diabetic pregnancies have an increased rate of fetal cardiomyopathy as compared to non-diabetic pregnancies. Read More

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http://dx.doi.org/10.1080/14767058.2020.1779213DOI Listing

[Gene edited animal models applied in human disease research].

Sheng Wu Gong Cheng Xue Bao 2020 May;36(5):849-860

College of Animal Science and Technology, Northwest Agriculture and Forestry University, Yangling 712100, Shaanxi, China.

Recently, with the development and the continuous improvement of various CRISPR systems represented by CRISPR/Cas9, gene editing technology has been gradually improved, and widely applied to the preparation of animal models of human diseases. The gene edited animal models provide important materials for the study of pathogenesis, pathological process, prevention and treatment of human diseases. At present, the gene edited animal models used in human disease research include mainly the rodent models represented by mice and rats, and large animal models represented by pigs. Read More

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http://dx.doi.org/10.13345/j.cjb.190395DOI Listing

Molecular Mechanisms of Adiponectin-Induced Attenuation of Mechanical Stretch-Mediated Vascular Remodeling.

Oxid Med Cell Longev 2020 21;2020:6425782. Epub 2020 May 21.

Department of Basic Sciences, College of Medicine QU Health, Qatar University, Doha, Qatar.

Hypertension induces vascular hypertrophy, which changes blood vessels structurally and functionally, leading to reduced tissue perfusion and further hypertension. It is also associated with dysregulated levels of the circulating adipokines leptin and adiponectin (APN). Leptin is an obesity-associated hormone that promotes vascular smooth muscle cell (VSMC) hypertrophy. Read More

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http://dx.doi.org/10.1155/2020/6425782DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7260649PMC

Identification of a novel titin-cap/telethonin mutation in a Portuguese family with hypertrophic cardiomyopathy.

Rev Port Cardiol 2020 Jun 18. Epub 2020 Jun 18.

Hospital da Luz - Inherited Cardiovascular Diseases & Hypertrophic Cardiomyopathy Center, Nova Medical School, Lisbon, Portugal.

Introduction And Objectives: Hypertrophic cardiomyopathy (HCM) is a genetically and phenotypically heterogeneous disease; there is still a large proportion of patients with no identified disease-causing mutation. Although the majority of mutations are found in the MYH7 and MYBPC3 genes, mutations in Z-disk-associated proteins have also been linked to HCM.

Methods: We assessed a small family with HCM based on family history, physical examination, 12-lead ECG, echocardiogram and magnetic resonance imaging. Read More

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http://dx.doi.org/10.1016/j.repc.2019.12.007DOI Listing

Contrast-Enhanced Echocardiographic Measurement of Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy: Comparison with Standard Echocardiography and Cardiac Magnetic Resonance.

J Am Soc Echocardiogr 2020 Jun 18. Epub 2020 Jun 18.

Inherited Cardiac Diseases Unit and Cardiovascular Imaging Laboratory, Vall d'Hebron University Hospital, Barcelona, Spain.

Background: Left ventricular wall thickness (LVWT) measurement is key in the diagnostic and prognostic assessment of hypertrophic cardiomyopathy (HCM). Recent investigations have highlighted discrepancies in LVWT by cardiac magnetic resonance (CMR) and standard echocardiography (S-Echo) in this condition. The aim of this study was to elucidate the role of contrast-enhanced echocardiography (C-Echo) to optimize LVWT measurement in patients with HCM. Read More

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http://dx.doi.org/10.1016/j.echo.2020.04.009DOI Listing
June 2020
4.056 Impact Factor

Temporally Coherent Cardiac Motion Tracking from Cine MRI: Traditional Registration Method and Modern CNN Method.

Med Phys 2020 Jun 21. Epub 2020 Jun 21.

Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.

Purpose: Cardiac motion tracking enables quantitative evaluation of myocardial strain, which is clinically interesting in cardiovascular disease research. However, motion tracking is difficult to perform manually. In this paper, we aim to develop and compare two fully automated motion tracking methods for the steady state free precession (SSFP) cine magnetic resonance imaging (MRI), and explore their use in real clinical scenario with different patient groups. Read More

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http://dx.doi.org/10.1002/mp.14341DOI Listing

Arrhythmic risk stratification by cardiac magnetic resonance tissue characterization: disclosing the arrhythmic substrate within the heart muscle.

Heart Fail Rev 2020 Jun 20. Epub 2020 Jun 20.

Cardiovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), University of Trieste, Via Pietro Valdoni 7, 34149, Trieste, Italy.

Sudden cardiac death (SCD) is a pivotal health problem worldwide. The identification of subjects at increased risk of SCD is crucial for the accurate selection of candidates for implantable cardioverter defibrillator (ICD) therapy. Current strategies for arrhythmic stratification largely rely on left ventricular (LV) ejection fraction (EF), mostly measured by echocardiography, and New York Heart Association functional status for heart failure with reduced EF. Read More

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http://dx.doi.org/10.1007/s10741-020-09986-0DOI Listing

Management of pediatric cardiac transplantation candidates with pulmonary hypertension and high pulmonary vascular resistance.

Turk J Pediatr 2020 ;62(3):461-467

Departments of Cardiovascular Surgery, Başkent University Faculty of Medicine, Ankara.

Background And Objectives: Right ventricular failure is an important cause of mortality and morbidity after orthotopic heart transplantation (OHT). The right ventricle of the donor may fail to accommodate to the high pulmonary vascular resistance (PVR) of the recipient. Pulmonary hypertension (PH) due to chronic heart failure with PVRi > 4 Wood units. Read More

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http://dx.doi.org/10.24953/turkjped.2020.03.013DOI Listing
January 2020

Atypical cardiac defects in patients with RASopathies: Updated data on CARNET study.

Birth Defects Res 2020 Jun;112(10):725-731

Pediatric Cardiology, Department of Pediatrics, Obstetrics and Gynecology, Sapienza University of Rome, Rome, Italy.

Background: RASopathies are a set of relatively common autosomal dominant clinically and genetically heterogeneous disorders. Cardiac outcomes in terms of mortality and morbidity for common heart defects (such as pulmonary valve stenosis and hypertrophic cardiomyopathy) have been reported. Nevertheless, also Atypical Cardiac Defects (ACDs) are described. Read More

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http://dx.doi.org/10.1002/bdr2.1670DOI Listing

Insight Into Myocardial Microstructure of Athletes and Hypertrophic Cardiomyopathy Patients Using Diffusion Tensor Imaging.

J Magn Reson Imaging 2020 Jun 18. Epub 2020 Jun 18.

Biomedical Imaging Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, Leeds Teaching Hospitals NHS Trust, Leeds, UK.

Background: Hypertrophic cardiomyopathy (HCM) remains the commonest cause of sudden cardiac death among young athletes. Differentiating between physiologically adaptive left ventricular (LV) hypertrophy observed in athletes' hearts and pathological HCM remains challenging. By quantifying the diffusion of water molecules, diffusion tensor imaging (DTI) MRI allows voxelwise characterization of myocardial microstructure. Read More

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http://dx.doi.org/10.1002/jmri.27257DOI Listing

Epicardial ultrasound in a case of myocardial bridge and apical hypertrophic cardiomyopathy.

J Card Surg 2020 Jun 19. Epub 2020 Jun 19.

Division of Cardiac Surgery, Department of Surgery, University of Virginia Health System, Charlottesville, Virginia.

A 59-year-old male with a history of unstable angina was diagnosed with a myocardial bridge of the left anterior descending artery (LAD) and apical variant hypertrophic cardiomyopathy (AHCM). He underwent unroofing of the myocardial bridge and a left ventricular apical myectomy. Intraoperatively, epicardial ultrasound was used to identify the myocardial bridge with systolic compression of the LAD and confirm resolution of this compression postoperatively. Read More

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http://dx.doi.org/10.1111/jocs.14627DOI Listing

Phosphorylation of cardiac myosin-binding protein-C contributes to calcium homeostasis.

J Biol Chem 2020 Jun 18. Epub 2020 Jun 18.

Internal Medicine, University of Cincinnati, United States.

Cardiac myosin-binding protein-C (cMyBP-C) is highly phosphorylated under basal conditions. However, its phosphorylation level is decreased in individuals with heart failure. The necessity of cMyBP-C phosphorylation for proper contractile function is well established, but the physiological and pathological consequences of decreased cMyBP-C phosphorylation in the heart are not clear. Read More

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http://dx.doi.org/10.1074/jbc.RA120.013296DOI Listing

Avoiding Burnout From Hypertrophic Cardiomyopathy.

J Am Coll Cardiol 2020 Jun;75(24):3044-3047

Vanderbilt University Medical Center, Division of Cardiovascular Medicine, Nashville, Tennessee.

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http://dx.doi.org/10.1016/j.jacc.2020.05.009DOI Listing

Outcomes in Patients With Hypertrophic Cardiomyopathy and Left Ventricular Systolic Dysfunction.

J Am Coll Cardiol 2020 Jun;75(24):3033-3043

HCM Institute, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts.

Background: End-stage (ES) hypertrophic cardiomyopathy (HCM) has been considered a particularly grim and unfavorable disease complication, associated with substantial morbidity and mortality, frequently requiring heart transplant. Previous reports have included small numbers of patients with relatively short follow-up, predominantly in prior treatment eras.

Objectives: The purpose of this study was to re-evaluate clinical profile and prognosis for end-stage heart failure in a large HCM cohort with contemporary management strategies. Read More

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http://dx.doi.org/10.1016/j.jacc.2020.04.045DOI Listing