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https://www.readbyqxmd.com/read/28548091/cellular-interplay-via-cytokine-hierarchy-causes-pathological-cardiac-hypertrophy-in-raf1-mutant-noonan-syndrome
#1
Jiani C Yin, Mathew J Platt, Xixi Tian, Xue Wu, Peter H Backx, Jeremy A Simpson, Toshiyuki Araki, Benjamin G Neel
Noonan syndrome (NS) is caused by mutations in RAS/ERK pathway genes, and is characterized by craniofacial, growth, cognitive and cardiac defects. NS patients with kinase-activating RAF1 alleles typically develop pathological left ventricular hypertrophy (LVH), which is reproduced in Raf1(L613V/+) knock-in mice. Here, using inducible Raf1(L613V) expression, we show that LVH results from the interplay of cardiac cell types. Cardiomyocyte Raf1(L613V) enhances Ca(2+) sensitivity and cardiac contractility without causing hypertrophy...
May 26, 2017: Nature Communications
https://www.readbyqxmd.com/read/28546762/cardiovascular-magnetic-resonance-imaging-clinical-implications-in-the-evaluation-of-connective-tissue-diseases
#2
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Genovefa Kolovou
Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28546535/a-novel-prkag2-mutation-in-a-chinese-family-with-cardiac-hypertrophy-and-ventricular-pre-excitation
#3
Kun-Qi Yang, Chao-Xia Lu, Ying Zhang, Yan-Kun Yang, Jia-Cheng Li, Tian Lan, Xu Meng, Peng Fan, Tao Tian, Lin-Ping Wang, Ya-Xin Liu, Xue Zhang, Xian-Liang Zhou
PRKAG2 syndrome is a rare autosomal dominant inherited disorder that is characterized by cardiac hypertrophy, ventricular pre-excitation and conduction system abnormalities. There is little knowledge in cardiovascular magnetic resonance (CMR) characteristics of PRKAG2 cardiomyopathy. This study investigated the genetic defect in a three-generation Chinese family with cardiac hypertrophy and ventricular pre-excitation using whole-exome sequencing. A novel missense mutation, c.1006 G > T (p.V336L), was identified in PRKAG2...
May 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28546358/autophagy-and-mitophagy-in-cardiovascular-disease
#4
REVIEW
José Manuel Bravo-San Pedro, Guido Kroemer, Lorenzo Galluzzi
Autophagy contributes to the maintenance of intracellular homeostasis in most cells of cardiovascular origin, including cardiomyocytes, endothelial cells, and arterial smooth muscle cells. Mitophagy is an autophagic response that specifically targets damaged, and hence potentially cytotoxic, mitochondria. As these organelles occupy a critical position in the bioenergetics of the cardiovascular system, mitophagy is particularly important for cardiovascular homeostasis in health and disease. Consistent with this notion, genetic defects in autophagy or mitophagy have been shown to exacerbate the propensity of laboratory animals to spontaneously develop cardiodegenerative disorders...
May 26, 2017: Circulation Research
https://www.readbyqxmd.com/read/28546113/stretch-activated-trpv2-channels-role-in-mediating-cardiopathies
#5
REVIEW
Elizabeth Aguettaz, Patrick Bois, Christian Cognard, Stéphane Sebille
Transient receptor potential vanilloid type 2, TRPV2, is a calcium-permeable cation channel belonging to the TRPV channel family. Although this channel has been first characterized as a noxious heat sensor, its mechanosensor property recently gained importance in various physiological functions. TRPV2 has been described as a stretch-mediated channel and a regulator of calcium homeostasis in several cell types and has been shown to be involved in the stretch-dependent responses in cardiomyocytes. Hence, several studies in the last years support the idea that TRPV2 play a key role in the function and structure of the heart, being involved in the cardiac compensatory mechanisms in response to pathologic or exercise-induced stress...
May 22, 2017: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/28545626/very-late-continued-reverse-remodelling-after-cardiac-resynchronization-therapy-in-patients-with-extreme-left-ventricular-dilatation
#6
Samuel Larue-Grondin, François Philippon, Jean-François Sarrazin, Sacha-Michelle Dubois-Sénéchal, Michelle Dubois, Mario Sénéchal
Response to cardiac resynchronization therapy (CRT) varies greatly among patients. We present 2 patients with severe heart failure symptoms (New York Heart Association class IV) and extreme initial left ventricular (LV) dilatation (LV end-diastolic diameter of 92 mm and 80 mm, respectively) and severe functional mitral regurgitation who underwent CRT device implantation. Long-term follow-up showed late (≥ 4 years) normalization of LV ejection fraction (LVEF), LV dimensions, and functional status. In a subgroup of patients with nonischemic dilated cardiomyopathy and complete left bundle branch block, late continued LV reverse remodelling may lead to normalization of LV volumes and LVEF and significant improvement in functional class...
June 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28545618/nosology-of-noncompaction-cardiomyopathy-the-emperor-still-wears-clothes
#7
EDITORIAL
Erwin Oechslin, Rolf Jenni
No abstract text is available yet for this article.
June 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28545449/quantifying-the-global-contribution-of-alcohol-consumption-to-cardiomyopathy
#8
Jakob Manthey, Sameer Imtiaz, Maria Neufeld, Margaret Rylett, Jürgen Rehm
BACKGROUND: The global impact of alcohol consumption on deaths due to cardiomyopathy (CM) has not been quantified to date, even though CM contains a subcategory for alcoholic CM with an effect of heavy drinking over time as the postulated underlying causal mechanism. In this feasibility study, a model to estimate the alcohol-attributable fraction (AAF) of CM deaths based on alcohol exposure measures is proposed. METHODS: A two-step model was developed based on aggregate-level data from 95 countries, including the most populous (data from 2013 or last available year)...
May 25, 2017: Population Health Metrics
https://www.readbyqxmd.com/read/28545398/advanced-glycation-end-products-in-children-with-type-1-diabetes-and-early-reduced-diastolic-heart-function
#9
Leif Brunvand, Martin Heier, Cathrine Brunborg, Kristian F Hanssen, Drude Fugelseth, Knut Haakon Stensaeth, Knut Dahl-Jørgensen, Hanna Dis Margeirsdottir
BACKGROUND: Reduced diastolic function is an early sign of diabetes cardiomyopathy in adults and is associated with elevated levels of HbA1c and advanced glycation end products (AGEs). OBJECTIVE: To assess the associations between early reduced diastolic function and elevated levels of HbA1c and AGEs in children and adolescents with type 1 diabetes (T1D). METHODS: One hundred fourty six T1D patients (age 8-18 years) without known diabetic complications were examined with tissue Doppler imaging and stratified into two groups according to diastolic function...
May 25, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28545369/impact-of-diabetes-on-cardiac-and-vascular-disease-role-of-calcium-signaling
#10
Tarik Smani, Isabel Gallardo-Castillo, Javier Ávila-Médina, Manuel F Jimenez-Navarro, Antonio Ordoñez, Abdelkrim Hmadcha
The pathophysiology linking diabetes and cardiovascular disease (CVD) is complex and multifactorial. The specific type of cardiomyopathy associated with diabetes, known as diabetic cardiomyopathy (DCM), is recognized as asymptomatic progression of structural and functional remodeling in the heart of diabetic patients in the absence of coronary atherosclerosis and hypertension. In other words, the presence of heart disease specifically in diabetic patients is also known as diabetic heart disease. This article reviews the impact of diabetes in heart and vascular beds focusing on molecular mechanisms involving the oxidative stress, the inflammation, the endothelium dysfunction and the alteration of the homeostasis of calcium, among others mechanisms...
May 23, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28544784/presynaptic-congenital-myasthenic-syndrome-with-a-homozygous-sequence-variant-in-lama5-combines-myopia-facial-tics-and-failure-of-neuromuscular-transmission
#11
Ricardo A Maselli, Juan Arredondo, Jessica Vázquez, Jessica X Chong, Michael J Bamshad, Deborah A Nickerson, Marian Lara, Fiona Ng, Victoria L Lo, Peter Pytel, Craig M McDonald
Defects in genes encoding the isoforms of the laminin alpha subunit have been linked to various phenotypic manifestations, including brain malformations, muscular dystrophy, ocular defects, cardiomyopathy, and skin abnormalities. We report here a severe defect of neuromuscular transmission in a consanguineous patient with a homozygous variant in the laminin alpha-5 subunit gene (LAMA5). The variant c.8046C>T (p.Arg2659Trp) is rare and has a predicted deleterious effect. The affected individual, who also carries a rare homozygous sequence variant in LAMA1, had muscle weakness, myopia, and facial tics...
May 25, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28543409/synergic-and-antagonistic-relationship-between-mmp-2-and-mmp-9-with-fibrosis-and-inflammation-in-chagas-cardiomyopathy
#12
REVIEW
Nayara I Medeiros, Juliana A S Gomes, Rodrigo Correa-Oliveira
Cardiomyopathy is the most important clinical manifestation in the chronic phase of Chagas disease because of its frequency, severity and impact on morbidity and mortality. The extracellular matrix degradation during cardiac remodeling in Trypanosoma cruzi infection is driven by matrix metalloproteinases (MMPs), primarily the MMP-2 and MMP-9 gelatinases. MMPs also regulate some molecules related to inflammation, such as growth factors, cytokines, and chemokines. The involvement of MMP-2 and MMP-9 is not yet fully understood in Chagas disease...
May 24, 2017: Parasite Immunology
https://www.readbyqxmd.com/read/28543399/baroreceptor-stimulation-in-a-patient-with-preexisting-subcutaneous-implantable-cardioverter-defibrillator
#13
Kay F Weipert, Jens Andrick, Ritvan Chasan, Christopher Gemein, Astrid Most, Christian W Hamm, Damir Erkapic, Joern Schmitt
BACKGROUND: Many patients with severe heart failure (HF) have an indication for baroreflex activation therapy (BAT) and an ICD. Concerns about device-device interactions were addressed in a study with small sample size that concluded combined BAT and ICD therapy is safe. There are no published data, however, concerning device-device interactions between BAT and a subcutaneous ICD (S-ICD). Since BAT frequently interferes with surface ECG recordings there are doubts about compatibility of BAT and S-ICD devices...
May 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28543396/functional-and-structural-assessment-of-the-effect-of-human-umbilical-cord-blood-mesenchymal-stem-cells-in-doxorubicin-induced-cardiotoxicity
#14
Somia H Abd Allah, Samia Hussein, Mai M Hasan, Raghda H A Deraz, Wafaa F Hussein, Laila M E Sabik
Cardiomyopathy induced by doxorubicin (DOX) was recognized at an early stage and also several years after drug administration. Mesenchymal stem cells (MSCs) have many properties that make them suitable for preventive and/ or regenerative therapies. In this study, we evaluated the effect of MSCs in the functional and the structural improvement of DOX-induced cardiomyopathy in rats. Ninety adult male albino rats were randomly divided into three equal groups of thirty rats each: Group I (control): rats received normal saline...
May 25, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28543170/specific-activation-of-cd4-cd8-double-negative-t-cells-by-t-cruzi-derived-glycolipids-induces-a-pro-inflammatory-profile-associated-with-cardiomyopathy-in-chagas-patients
#15
Lívia Silva Araújo Passos, Luísa Mourão Dias Magalhães, Rodrigo Pedro Soares, Alexandre F Marques, Maria do Carmo Pereira Nunes, Kenneth J Gollob, Walderez Ornelas Dutra
Cardiomyopathy is the most severe outcome of Chagas disease, causing over 12,000 deaths/year. Immune cells participate in cardiomyopathy development either by direct tissue destruction, or by driving inflammation. We have shown that CD4-CD8- (double negative - DN) T cells are major sources of inflammatory and anti-inflammatory cytokines, associated with the cardiac (CARD) and indeterminate (IND) forms of Chagas disease, respectively. Here, we sought to identify Trypanosoma cruzi derived components that lead to activation of DN T cells in Chagas patients...
May 24, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28543134/-real-life-longevity-of-implantable-cardioverter-defibrillator-devices
#16
Antonis S Manolis, Themistoklis Maounis, Spyridon Koulouris, Vassilios Vassilikos
BACKGROUND: Manufacturers of implantable cardioverter-defibrillators (ICDs) promise a 5- to 9-year projected longevity; however, real-life data indicate otherwise. The aim of the present study was to assess ICD longevity among 685 consecutive patients over the last 20 years. HYPOTHESIS: Real-life longevity of ICDs may differ from that stated by the manufacturers. METHODS: The study included 601 men and 84 women (mean age, 63.1 ± 13.3 years)...
May 22, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28543099/evidence-of-altered-mitochondrial-protein-expression-after-chronic-ethanol-consumption-in-the-aged-estrogen-deficient-female-rat-heart
#17
Alexandra M Garvin, J L Miller-Lee, D R Sharda, G M Kanski, J C Hunter, Donna H Korzick
BACKGROUND: Estrogen loss has been implicated to increase the risk of alcoholic cardiomyopathy in post-menopausal women. The purpose of this study was to identify novel mitochondrial protein targets for the treatment of alcoholic cardiomyopathy in aged women using a state-of-the-art proteomics approach. We hypothesized that chronic ethanol (EtOH) ingestion exacerbates maladaptive mitochondrial protein expression in the aged female heart METHODS: Adult (3 mo) and aged (18 mo) F344 ovary-intact or ovariectomized (OVX) rats were randomly assigned an EtOH or control Lieber-DeCarli 'all liquid' diet for 20 wks...
May 19, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28543059/natural-pathology-of-the-captive-chimpanzee-pan-troglodytes-a-35-year-review
#18
REVIEW
Shyamesh Kumar, Hannah Laurence, Michael A Owston, R Mark Sharp, Priscilla Williams, Robert E Lanford, Gene B Hubbard, Edward J Dick
We present the spontaneous pathological lesions identified as a result of necropsy or biopsy for 245 chimpanzees (Pan troglodytes) over a 35-year period. A review of the pathology database was performed for all diagnoses on chimpanzees from 1980 to 2014. All morphologic diagnoses, associated system, organ, etiology, and demographic information were reviewed and analyzed. Cardiomyopathy was the most frequent lesion observed followed by hemosiderosis, hyperplasia, nematodiasis, edema, and hemorrhage. The most frequently affected systems were the gastrointestinal, cardiovascular, urogenital, respiratory, and lymphatic/hematopoietic systems...
May 23, 2017: Journal of Medical Primatology
https://www.readbyqxmd.com/read/28542596/progressive-mitochondrial-protein-lysine-acetylation-and-heart-failure-in-a-model-of-friedreich-s-ataxia-cardiomyopathy
#19
Amanda R Stram, Gregory R Wagner, Brian D Fogler, P Melanie Pride, Matthew D Hirschey, R Mark Payne
INTRODUCTION: The childhood heart disease of Friedreich's Ataxia (FRDA) is characterized by hypertrophy and failure. It is caused by loss of frataxin (FXN), a mitochondrial protein involved in energy homeostasis. FRDA model hearts have increased mitochondrial protein acetylation and impaired sirtuin 3 (SIRT3) deacetylase activity. Protein acetylation is an important regulator of cardiac metabolism and loss of SIRT3 increases susceptibility of the heart to stress-induced cardiac hypertrophy and ischemic injury...
2017: PloS One
https://www.readbyqxmd.com/read/28541593/clinical-and-prognostic-relevancy-of-left-ventricular-trabeculation-assessed-by-cardiac-magnetic-resonance-in-patients-with-dilated-cardiomyopathy
#20
Łukasz Mazurkiewicz, Joanna Petryka, Mateusz Śpiewak, Barbara Miłosz-Wieczorek, Łukasz A Małek, Anna Jasińska, Ewelina Jarmus, Magdalena Marczak, Jolanta Misko, Jacek Grzybowski
BACKGROUND AND AIM: We sought to search for factors that are associated with the magnitude of trabeculation by cardiac magnetic resonance (CMR), and evaluate the impact of trabeculations on outcomes in patients with dilated cardiomyopathy (DCM). METHODS: We evaluated clinical profiles and outcomes of 276 subjects with DCM (age - 33,2+/-13,3 years, 160 males). Trabeculation was quantified as trabeculated/total myocardial mass ratio (TM/M). Subjects were stratified into 3 subgroups (A, B, and C) according to the tertiles of rising TM/M values (33% ranges)...
May 25, 2017: Kardiologia Polska
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