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Hypertrophe cardiomyopathy

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https://www.readbyqxmd.com/read/30552684/catecholamine-response-to-exercise-in-patients-with-non-obstructive-hypertrophic-cardiomyopathy
#1
Ankit B Shah, Mary Z Bechis, Marcel Brown, Jennifer Michaud Finch, Garrett Loomer, Erich Groezinger, Rory B Weiner, Meagan M Wasfy, Michael H Picard, Michael A Fifer, Gregory B Lewis, Aaron L Baggish
KEY POINTS SUMMARY: Intense physical activity, a potent stimulus for sympathetic nervous system activation, is thought to increase the risk of malignant ventricular arrhythmias among patients with hypertrophic cardiomyopathy (HCM). As a result, the majority of patients with HCM deliberately reduce their habitual physical activity after diagnosis and this lifestyle change puts them at risk for sequelae of a sedentary lifestyle; weight gain, hypertension, hyperlipidemia, insulin resistance, coronary artery disease and increased morbidity and mortality...
December 15, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/30551401/eets-ppars-activation-together-mediates-the-preventive-effect-of-naringenin-in-high-glucose-induced-cardiomyocyte-hypertrophy
#2
Jie Zhang, Hongmei Qiu, Jiajun Huang, Shumei Ding, Bo Huang, Ping Zhou, Qingsong Jiang
BACKGROUND: Cardiac hypertrophy is a key pathological process in the context of diabetic cardiomyopathy. Naringenin exhibits multiple pharmacological activities, but the effect of naringenin on cardiomyocyte hypertrophy under diabetic conditions is still far from clear. METHODS: Cardiomyocyte hypertrophy was induced by high glucose (HG, glucose at 25.5 mmol/L) in H9c2 cells, which was determined by cell surface area, protein content and atrial natriuretic factor (ANF) mRNA expression...
January 2019: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/30550750/mybpc3-truncation-mutations-enhance-actomyosin-contractile-mechanics-in-human-hypertrophic-cardiomyopathy
#3
Thomas S O'Leary, Julia Snyder, Sakthivel Sadayappan, Sharlene M Day, Michael J Previs
RATIONALE: Truncation mutations in the MYBPC3 gene, encoding for cardiac myosin-binding protein C (MyBP-C), are the leading cause of hypertrophic cardiomyopathy (HCM). Whole heart, fiber and molecular studies demonstrate that MyBP-C is a potent modulator of cardiac contractility, but how these mutations contribute to HCM is unresolved. OBJECTIVES: To readdress whether MYBPC3 truncation mutations result in loss of MyBP-C content and/or the expression of truncated MyBP-C from the mutant allele and determine how these mutations effect myofilament sliding in human myocardium...
December 11, 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/30548699/hypertrophic-cardiomyopathy-with-dynamic-obstruction-and-high-left-ventricular-outflow-gradients-associated-with-paradoxical-apical-ballooning
#4
Mark V Sherrid, Katherine Riedy, Barry Rosenzweig, Monica Ahluwalia, Milla Arabadjian, Muhamed Saric, Sandhya Balaram, Daniel G Swistel, Harmony R Reynolds, Bette Kim
BACKGROUND: Acute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability. METHODS, RESULTS: We searched for acute LV ballooning with apical hypokinesia/akinesia in databases of two HCM treatment programs. Diagnosis of OHCM was made by conventional criteria of LV hypertrophy in the absence of a clinical cause for hypertrophy and mitral-septal contact...
December 12, 2018: Echocardiography
https://www.readbyqxmd.com/read/30548303/lncrna-miat-regulates-fibrosis-in-hypertrophic-cardiomyopathy-hcm-by-mediating-the-expression-of-mir-29a-3p
#5
Jing Zhou, Yu Zhou, Cong-Xia Wang
AIM: This study aimed to investigate the molecular mechanism underlying the fibrosis in hypertrophic cardiomyopathy (HCM). METHOD: Quantitative real-time polymerase chain reaction (qRT-PCR) was performed to measure the expression of potentially relevant microRNAs (miRNAs) and long noncoding RNAs (lncRNAs) in patients with HCM suffering from fibrosis and patients with HCM free of fibrosis. In addition, the regulatory relationship between lncRNAs and miR-29a was studied using a luciferase assay...
December 11, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/30547297/transient-ischemic-dilation-in-hypertrophic-cardiomyopathy-a-complex-sign-in-a-complex-disease
#6
EDITORIAL
Roberto Sciagrà
No abstract text is available yet for this article.
December 13, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/30545447/septal-reduction-therapy-for-hypertrophic-obstructive-cardiomyopathy
#7
EDITORIAL
Michael A Fifer
No abstract text is available yet for this article.
December 18, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/30545446/survival-after-alcohol-septal-ablation-in-patients-with-hypertrophic-obstructive-cardiomyopathy
#8
Angelika Batzner, Barbara Pfeiffer, Anna Neugebauer, Diaa Aicha, Christoph Blank, Hubert Seggewiss
BACKGROUND: Alcohol-induced infarction for treatment of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) was discussed as a risk factor for increased cardiac mortality during follow-up. OBJECTIVES: This study sought to report on long-term survival after echo-guided alcohol septal ablation (percutaneous transluminal septal myocardial ablation [PTSMA]) in symptomatic patients with HOCM. METHODS: Between May 2000 and June 2017, PTSMA with alcohol injection was performed in 952 patients (age 55...
December 18, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/30542921/contemporary-outcomes-of-pediatric-restrictive-cardiomyopathy-a-single-center-experience
#9
Samuel G Wittekind, Thomas D Ryan, Zhiqian Gao, Farhan Zafar, Richard J Czosek, Clifford W Chin, John L Jefferies
BACKGROUND: Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx. METHODS: All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated...
December 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/30535072/clinical-presentation-and-survival-of-childhood-hypertrophic-cardiomyopathy-a-retrospective-study-in-united-kingdom
#10
Gabrielle Norrish, Ella Field, Karen Mcleod, Maria Ilina, Graham Stuart, Vinay Bhole, Orhan Uzun, Elspeth Brown, Piers E F Daubeney, Amrit Lota, Katie Linter, Sujeev Mathur, Tara Bharucha, Khoon Li Kok, Satish Adwani, Caroline B Jones, Zdenka Reinhardt, Juan Pablo Kaski
Aims: Understanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort. Methods and results: Six hundred and eighty-seven patients with HCM presented at a median age of 5...
December 6, 2018: European Heart Journal
https://www.readbyqxmd.com/read/30531895/analysis-of-51-proposed-hypertrophic-cardiomyopathy-genes-from-genome-sequencing-data-in-sarcomere-negative-cases-has-negligible-diagnostic-yield
#11
Kate L Thomson, Elizabeth Ormondroyd, Andrew R Harper, Tim Dent, Karen McGuire, John Baksi, Edward Blair, Paul Brennan, Rachel Buchan, Teofila Bueser, Carolyn Campbell, Gerald Carr-White, Stuart Cook, Matthew Daniels, Sri V V Deevi, Judith Goodship, Jesse B G Hayesmoore, Alex Henderson, Teresa Lamb, Sanjay Prasad, Paula Rayner-Matthews, Leema Robert, Linda Sneddon, Hannah Stark, Roddy Walsh, James S Ware, Martin Farrall, Hugh C Watkins
PURPOSE: Increasing numbers of genes are being implicated in Mendelian disorders and incorporated into clinical test panels. However, lack of evidence supporting the gene-disease relationship can hinder interpretation. We explored the utility of testing 51 additional genes for hypertrophic cardiomyopathy (HCM), one of the most commonly tested Mendelian disorders. METHODS: Using genome sequencing data from 240 sarcomere gene negative HCM cases and 6229 controls, we undertook case-control and individual variant analyses to assess 51 genes that have been proposed for HCM testing...
December 11, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/30527518/texture-analysis-of-cardiovascular-magnetic-resonance-cine-images-differentiates-aetiologies-of-left-ventricular-hypertrophy
#12
R Schofield, B Ganeshan, M Fontana, A Nasis, S Castelletti, S Rosmini, T A Treibel, C Manisty, R Endozo, A Groves, J C Moon
AIM: To investigate whether unenhanced cardiovascular magnetic resonance (CMR) balanced steady state free precession (bSSFP) cine images could be analysed using textural analysis (TA) software to differentiate different aetiologies of disease causing increased myocardial wall thickness (left ventricular hypertrophy [LVH]) and indicate the severity of myocardial tissue abnormality. MATERIALS AND METHODS: A mid short axis unenhanced cine frame of 216 patients comprising 50 cases of hypertrophic cardiomyopathy (HCM; predominantly Left ventricular outflow tract obstruction [LVOTO] subtype), 52 cases of cardiac amyloid (CA; predominantly AL: light chain subtype), 68 cases of aortic stenosis (AS), 15 hypertensive patients with LVH (HTN+LVH), and 31 healthy volunteers (HV) underwent TA of the CMR cine images (CMRTA) using TexRAD (TexRAD Ltd, Cambridge, UK)...
October 24, 2018: Clinical Radiology
https://www.readbyqxmd.com/read/30522606/pulsed-doppler-echocardiography-an-historical-perspective
#13
Julius M Gardin
Over the past six decades, echocardiography has evolved into an important technique for not only imaging cardiac structures, but also, by employing the Doppler equation, for assessing cardiac blood flow and tissue velocities. This review focuses on pulsed Doppler echocardiography: its principles, early development, and clinical applications. Important clinical applications include: (1) measurement of flow velocities, stroke volumes, and regurgitant and shunt volumes; (2) assessment of time intervals, e.g., pulmonary artery acceleration time as a measure of pulmonary artery pressure and resistance or the timing of mitral regurgitation in hypertrophic cardiomyopathy; (3) detection of turbulent flow in regurgitation, stenoses, and shunts, enhanced by the implementation of color Doppler; and (4) evaluation of left ventricular diastolic function in conjunction with pulsed tissue Doppler and deformation (strain) measurements...
December 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/30521840/mechanistic-role-of-the-creb-regulated-transcription-coactivator-1-in-cardiac-hypertrophy
#14
Karoline Morhenn, Thomas Quentin, Helen Wichmann, Michael Steinmetz, Maksymilian Prondzynski, Klaus-Dieter Söhren, Torsten Christ, Birgit Geertz, Sabine Schröder, Friedrich A Schöndube, Gerd Hasenfuss, Saskia Schlossarek, Wolfram H Zimmermann, Lucie Carrier, Thomas Eschenhagen, Jean-René Cardinaux, Susanne Lutz, Elke Oetjen
The sympathetic nervous system is the main stimulator of cardiac function. While acute activation of the β-adrenoceptors exerts positive inotropic and lusitropic effects by increasing cAMP and Ca2+ , chronically enhanced sympathetic tone with changed β-adrenergic signaling leads to alterations of gene expression and remodeling. The CREB-regulated transcription coactivator 1 (CRTC1) is activated by cAMP and Ca2+ . In the present study, the regulation of CRTC1 in cardiomyocytes and its effect on cardiac function and growth was investigated...
December 4, 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/30511546/a-novel-mutation-of-the-myh7-gene-in-a-patient-with-hypertrophic-cardiomyopathy
#15
Nicholas Goel, Charles B Huddleston, Andrew C Fiore
Goel N, Huddleston CB, Fiore AC. A novel mutation of the MYH7 gene in a patient with hypertrophic cardiomyopathy. Turk J Pediatr 2018; 60: 315-318. Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by asymmetric cardiac hypertrophy due to inherited mutations in genes that encode sarcomeric proteins. MYH7, which encodes β-myosin heavy chain, is among the most commonly mutated genes in patients affected by HCM. We aimed to identify the specific mutation responsible for HCM in a six-month old Caucasian patient...
2018: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/30508933/successful-treatment-of-drug-induced-esophageal-ulcer-in-a-patient-with-chronic-heart-failure-a-case-report
#16
Kazuki Maesaka, Yoshiki Tsujii, Shinichiro Shinzaki, Shunsuke Yoshii, Yoshito Hayashi, Hideki Iijima, Kei Nakamoto, Tomohito Ohtani, Yasushi Sakata, Tetsuo Takehara
RATIONALE: Although esophageal compression due to cardiomegaly may be a risk factor of drug-induced esophageal injuries (DIEIs), the causal relationship between the two conditions has not been fully demonstrated. PATIENT CONCERNS: We present a case of a drug-induced esophageal ulcer caused by left atrial enlargement in a 44-year-old woman with end-stage hypertrophic cardiomyopathy. Upper gastrointestinal endoscopy showed a deep, circumferential ulcer in the middle thoracic esophagus...
November 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/30508693/development-of-induced-pluripotent-stem-cells-from-a-patient-with-hypertrophic-cardiomyopathy-who-carries-the-pathogenic-myosin-heavy-chain-7-mutation-p-arg403gln
#17
Mira Holliday, Samantha Barratt Ross, Seakcheng Lim, Melissa Mangala, Adam Hill, Henrietta Cserne Szappanos, Livia Hool, Christopher Semsarian
Hypertrophic cardiomyopathy (HCM) is an inherited cardiomyopathy characterized by left ventricular hypertrophy ≥15 mm in the absence of loading conditions. HCM has a prevalence of up to one in 200, and can result in significant adverse outcomes including heart failure and sudden cardiac death. An induced pluripotent stem cell (iPSC) line was generated from peripheral blood mononuclear cells obtained from the whole blood of a 38-year-old female patient with HCM in which genetic testing identified the well-known pathogenic p...
November 28, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/30508596/a-positive-feedback-regulation-of-heme-oxygenase-1-by-celf1-in-cardiac-myoblast-cells
#18
Yang Liu, Huiwen Wang, Jun Wang, Bin Wei, Xinyi Zhang, Mengqi Zhang, Dong Cao, Jiang Dai, Zhen Wang, Eric Nyirimigabo, Guangju Ji
As an RNA binding protein, CUG-BP Elav-like family (CELF) has been shown to be critical for heart biological functions. However, no reports have revealed the function of CELF1 in hypertrophic cardiomyopathy (HCM). Hinted by RNA immunoprecipitation-sequencing (RIP-seq) data, the influence of the CELF protein on heme oxygenase-1 (HO-1) expression was tested by modulating CELF1 levels. Cardiac hypertrophy is related to oxidative stress-induced damage. Hence, the cardiovascular system may be protected against further injury by upregulating the expression of antioxidant enzymes, such as HO-1...
November 30, 2018: Biochimica et biophysica acta. Gene regulatory mechanisms
https://www.readbyqxmd.com/read/30508591/right-ventricular-involvement-in-hypertrophic-cardiomyopathy-patterns-and-implications
#19
REVIEW
Kalliopi Keramida, George Lazaros, Petros Nihoyannopoulos
Although hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy worldwide, the criteria for its definition and most of the literature concern the left ventricle, confirming the theory that the right ventricle is the neglected one. Right ventricular (RV) involvement includes structural and functional changes with significant impact on clinical presentation and prognosis. The pattern of RV hypertrophy can be variable with possible dynamic obstruction. Histological findings suggest similar pathogenetic changes in both ventricles supporting the common myopathic process with sarcomeric mutations...
November 30, 2018: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/30504773/cardiac-specific-prmt1-ablation-causes-heart-failure-through-camkii-dysregulation
#20
Jung-Hoon Pyun, Hyun-Ji Kim, Myong-Ho Jeong, Byeong-Yun Ahn, Tuan Anh Vuong, Dong I Lee, Seri Choi, Seung-Hoi Koo, Hana Cho, Jong-Sun Kang
Dysregulation of Ca2+ /calmodulin-dependent protein kinase (CaMK)II is closely linked with myocardial hypertrophy and heart failure. However, the mechanisms that regulate CaMKII activity are incompletely understood. Here we show that protein arginine methyltransferase 1 (PRMT1) is essential for preventing cardiac CaMKII hyperactivation. Mice null for cardiac PRMT1 exhibit a rapid progression to dilated cardiomyopathy and heart failure within 2 months, accompanied by cardiomyocyte hypertrophy and fibrosis. Consistently, PRMT1 is downregulated in heart failure patients...
November 30, 2018: Nature Communications
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