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https://www.readbyqxmd.com/read/28432072/translocase-of-inner-membrane-50-functions-as-a-novel-protective-regulator-of-pathological-cardiac-hypertrophy
#1
Kai Tang, Yifan Zhao, Hailing Li, Mengyun Zhu, Weiming Li, Weijing Liu, Guofu Zhu, Dachun Xu, Wenhui Peng, Ya-Wei Xu
BACKGROUND: Translocase of inner membrane 50 (TIM50) is a member of the translocase of inner membrane (TIM) complex in the mitochondria. Previous research has demonstrated the role of TIM50 in the regulation of oxidative stress and cardiac morphology. However, the role of TIM50 in pathological cardiac hypertrophy remains unknown. METHODS AND RESULTS: In the present study we found that the expression of TIM50 was downregulated in hypertrophic hearts. Using genetic loss-of-function animal models, we demonstrated that TIM50 deficiency increased heart and cardiomyocyte size with more severe cardiac fibrosis compared with wild-type littermates...
April 21, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28431061/high-prevalence-of-arrhythmic-and-myocardial-complications-in-patients-with-cardiac-glycogenosis-due-to-prkag2-mutations
#2
Julien Thevenon, Gabriel Laurent, Flavie Ader, Pascal Laforêt, Didier Klug, Anju Duva Pentiah, Laurent Gouya, Claude Alain Maurage, Salem Kacet, Jean-Christophe Eicher, Juliette Albuisson, Michel Desnos, Eric Bieth, Denis Duboc, Laurent Martin, Patricia Réant, François Picard, Claire Bonithon-Kopp, Elodie Gautier, Christine Binquet, Christel Thauvin-Robinet, Laurence Faivre, Patrice Bouvagnet, Philippe Charron, Pascale Richard
Aims: Mutations in PRKAG2, the gene encoding for the γ2 subunit of 5'-AMP-activated protein kinase (AMPK), are responsible for an autosomal dominant glycogenosis with a cardiac presentation, associating hypertrophic cardiomyopathy (HCM), ventricular pre-excitation (VPE), and progressive heart block. The aim of this study was to perform a retrospective time-to-event study of the clinical manifestations associated with PRKAG2 mutations. Methods and Results: A cohort of 34 patients from 9 families was recruited between 2001 and 2010...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#3
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28428073/isosteviol-prevents-the-prolongation-of-action-potential-in-hypertrophied-cardiomyoctyes-by-regulating-transient-outward-potassium-and-l-type-calcium-channels
#4
Zhuo Fan, Nanying Lv, Xiao Luo, Wen Tan
Cardiac hypertrophy is a thickening of the heart muscle that is associated with cardiovascular diseases such as hypertension and myocardial infarction. It occurs initially as an adaptive process against increased workloads and often leads to sudden arrhythmic deaths. Studies suggest that the lethal arrhythmia is attributed to hypertrophy-induced destabilization of cardiac electrical activity, especially the prolongation of the action potential. The reduced activity of Ito is demonstrated to be responsible for the ionic mechanism of prolonged action potential duration and arrhythmogeneity...
April 17, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28426781/tauroursodeoxycholic-acid-tudca-attenuates-pressure-overload-induced-cardiac-remodeling-by-reducing-endoplasmic-reticulum-stress
#5
Shilpa Rani, Pradeep Kumar Sreenivasaiah, Jin Ock Kim, Mi Young Lee, Wan Seok Kang, Yong Sook Kim, Youngkeun Ahn, Woo Jin Park, Chunghee Cho, Do Han Kim
Pressure overload in the heart induces pathological hypertrophy and is associated with cardiac dysfunction. Apoptosis and fibrosis signaling initiated by the endoplasmic reticulum stress (ERS) is known to contribute to these maladaptive effects. The aim of this study was to investigate whether reduction of ERS by a known chemical chaperone, tauroursodeoxycholic acid (TUDCA) can attenuate pressure overload-induced cardiac remodeling in a mouse model of transverse aortic constriction (TAC). Oral administration of TUDCA at a dose of 300 mg/kg body weight (BW) in the TUDCA-TAC group reduced ERS markers (GRP78, p-PERK, and p-eIf2α), compared to the Vehicle (Veh)-TAC group...
2017: PloS One
https://www.readbyqxmd.com/read/28424545/efficacy-and-safety-of-alcohol-septal-ablation-in-patients-over-65-years-old-with-obstructive-hypertrophic-cardiomyopathy
#6
Laila Cheddadi, Olivier Lairez, Thibault Lhermusier, Francisco Campelo-Parada, Michel Galinier, Didier Carrié, Nicolas Boudou
BACKGROUND: The performance of alcohol septal ablation (ASA) in elderly symptomatic patients with drug-refractory obstructive hypertrophic cardiomyopathy is still to be confirmed. The objective of this study was to compare the efficacy and safety of ASA in patients under and over 65 years old. METHODS AND RESULTS: Fifty-one consecutive patients with obstructive hypertrophic cardio-myopathy who underwent ASA were retrospectively included and reviewed for in-hospital major acute cardiac events and follow-up...
2017: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/28421174/utility-of-cardiovascular-magnetic-resonance-derived-wave-intensity-analysis-as-a-marker-of-ventricular-function-in-children-with-heart-failure-and-normal-ejection-fraction
#7
Hopewell N Ntsinjana, Robin Chung, Paolo Ciliberti, Vivek Muthurangu, Silvia Schievano, Jan Marek, Kim H Parker, Andrew M Taylor, Giovanni Biglino
OBJECTIVE: This study sought to explore the diagnostic insight of cardiovascular magnetic resonance (CMR)-derived wave intensity analysis to better study systolic dysfunction in young patients with chronic diastolic dysfunction and preserved ejection fraction (EF), comparing it against other echocardiographic and CMR parameters. BACKGROUND: Evaluating systolic and diastolic dysfunctions in children is challenging, and a gold standard method is currently lacking...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28420666/prevalence-and-clinical-implication-of-double-mutations-in-hypertrophic-cardiomyopathy-revisiting-the-gene-dose-effect
#8
Dana Fourey, Melanie Care, Katherine A Siminovitch, Adaya Weissler-Snir, Waseem Hindieh, Raymond H Chan, Michael H Gollob, Harry Rakowski, Arnon Adler
BACKGROUND: Available data suggests that double mutations in patients with hypertrophic cardiomyopathy are not rare and are associated with a more severe phenotype. Most of this data, however, is based on noncontemporary variant classification. METHODS AND RESULTS: Clinical data of all hypertrophic cardiomyopathy patients with 2 rare genetic variants were retrospectively reviewed and compared with a group of patients with a single disease-causing variant. Furthermore, a literature search was performed for all studies with information on prevalence and outcome of patients with double mutations...
April 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28418355/-morphometric-features-of-cardiomyocytes-in-the-ventricular-septum-of-patients-with-hypertrophic-cardiomyopathy
#9
I F Egorova, R A Serov, L A Bockeria
AIM: to determine the diameter and length of cardiomyocytes (CMC) in the ventricular septum (VS) of patients with hypertrophic cardiomyopathy (HCM) and to analyze correlations of a change in CMC sizes with the anatomical features of the heart in the patients. MATERIAL AND METHODS: Longitudinal sections of intraoperative myocardial biopsy specimens taken from 23 patients aged 15-59 years with HCM were treated using immunohistochemical detection of connexin 43; the sizes of 50 CMCs were measured; a 4-point scale was used to assess the degree of myofibril loss (MFL) in these cells...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28413114/left-atrial-appendage-thrombi-relate-to-easily-accessible-clinical-parameters-in-patients-undergoing-atrial-fibrillation-transcatheter-ablation-a-multicenter-study
#10
Matteo Anselmino, Lucia Garberoglio, Sebastiano Gili, Emanuele Bertaglia, Giuseppe Stabile, Raffaella Marazzi, Sakis Themistoclakis, Franceso Solimene, Simone Frea, Walter Grosso Marra, Mara Morello, Marco Scaglione, Roberto De Ponti, Fiorenzo Gaita
BACKGROUND: Transesophageal echocardiography (TEE) is routinely performed before atrial fibrillation (AF) transcatheter ablation to exclude the presence of left atrial (LA) or LA appendage (LAA) thrombi. The aim of the study is to evaluate if easily accessible clinical parameters may relate to the presence of LA or LAA thrombi to identify patients who could potentially avoid TEE. METHODS AND RESULTS: Between January 2012 and September 2014, data from 1539 consecutive patients undergoing TEE, as a work-up before AF transcatheter ablation, in six large volume centers were collected...
April 8, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28413031/matrix-metalloproteinases-in-remodeling-of-lower-extremity-veins-and-chronic-venous-disease
#11
Yunfei Chen, Wei Peng, Joseph D Raffetto, Raouf A Khalil
The veins of the lower extremity are equipped with efficient wall, contractile vascular smooth muscle (VSM), and competent valves in order to withstand the high venous hydrostatic pressure in the lower limb and allow unidirectional movement of deoxygenated blood toward the heart. The vein wall structure and function are in part regulated by matrix metalloproteinases (MMPs). MMPs are zinc-dependent endopeptidases that are secreted as inactive pro-MMPs by different cells in the venous wall including fibroblasts, VSM, and leukocytes...
2017: Progress in Molecular Biology and Translational Science
https://www.readbyqxmd.com/read/28412414/endothelin-1-promotes-hypertrophic-remodelling-of-cardiac-myocytes-by-activating-sustained-signalling-and-transcription-downstream-of-endothelin-type-a-receptors
#12
Caroline R Archer, Emma L Robinson, Faye M Drawnel, H Llewelyn Roderick
G-protein coupled receptor (GPCR) mediated activation of the MAPK signalling cascade is a key pathway in the induction of hypertrophic remodelling of the heart - a response to pathological cues including hypertension and myocardial infarction. While levels of pro-hypertrophic hormone agonists of GPCRs increase during periods of greater workload to enhance cardiac output, hypertrophy does not necessarily result. Here we investigated the relationship between the duration of exposure to the pro-hypertrophic GPCR agonist endothelin-1 (ET-1) and the induction of hypertrophic remodelling in neonatal rat ventricular myocytes (NRVM) and in the adult rat heart in vivo...
April 13, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/28403507/endothelial-dysfunction-and-mechanobiology-in-pathological-cutaneous-scarring-lessons-learned-from-soft-tissue-fibrosis
#13
REVIEW
C Huang, L Liu, Z You, Y Zhao, J Dong, Y Du, R Ogawa
Hypertrophic scars/keloids are pathogenic scars that are characterized by collagen and fibroblast accumulation. The endothelia in the lesions are mechanosensitive and participate actively in the pathogenesis of these scars. The present review summarizes how endothelium responds to mechanical stimuli such as shear, stretch, and hydrostatic pressure. It also shows that in heart, liver, kidney, and lung fibrosis, endothelial dysfunctions (EDs) associate with changes in vascular tone, endothelial permeability, coagulation, and vasomodulation that result in inflammation, hypertension, and coagulation...
April 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28402392/caveolae-localized-l-type-ca2-channels-do-not-contribute-to-function-or-hypertrophic-signalling-in-the-mouse-heart
#14
Robert N Correll, Catherine A Makarewich, Hongyu Zhang, Chen Zhang, Michelle A Sargent, Allen J York, Remus M Berretta, Xiongwen Chen, Steven R Houser, Jeffery D Molkentin
Aims: L-type Ca2+ channels (LTCCs) in adult cardiomyocytes are localized to t-tubules where they initiate excitation-contraction coupling. Our recent work has shown that a subpopulation of LTCCs found at the surface sarcolemma in caveolae of adult feline cardiomyocytes can also generate a Ca2+ microdomain that activates nuclear factor of activated T-cells signaling and cardiac hypertrophy, although the relevance of this paradigm to hypertrophy regulation in vivo has not been examined...
April 11, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28402037/atrial-electrophysiological-and-molecular-remodelling-induced-by-obstructive-sleep-apnoea
#15
Devika Channaveerappa, Jacob C Lux, Kelly L Wormwood, Timothy A Heintz, Meredith McLerie, Jacqueline A Treat, Hannah King, Donia Alnasser, Robert J Goodrow, Glenn Ballard, Robert Decker, Costel C Darie, Brian K Panama
Obstructive sleep apnoea (OSA) affects 9-24% of the adult population. OSA is associated with atrial disease, including atrial enlargement, fibrosis and arrhythmias. Despite the link between OSA and cardiac disease, the molecular changes in the heart which occur with OSA remain elusive. To study OSA-induced cardiac changes, we utilized a recently developed rat model which closely recapitulates the characteristics of OSA. Male Sprague Dawley rats, aged 50-70 days, received surgically implanted tracheal balloons which were inflated to cause transient airway obstructions...
April 12, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28399117/disproportionate-cardiac-hypertrophy-during-early-postnatal-development-in-infants-born-preterm
#16
Christina Y L Aye, Adam J Lewandowski, Pablo Lamata, Ross Upton, Esther Davis, Eric O Ohuma, Yvonne Kenworthy, Henry Boardman, Samuel Wopperer, Alice Packham, Satish Adwani, Kenny McCormick, Aris T Papageorghiou, Paul Leeson
BACKGROUND: Adults born very preterm have increased cardiac mass and reduced function. We investigated whether a hypertrophic phenomenon occurs in later preterm infants and when this occurs during early development. METHODS: Cardiac ultrasound was performed on 392 infants (33% preterm at mean gestation 34+/-2 weeks). Scans were performed during fetal development in 137, at birth and three months postnatal age in 200 and during both fetal and postnatal development in 55...
April 11, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28396501/mechanism-of-progressive-heart-failure-and-significance-of-pulmonary-hypertension-in-obstructive-hypertrophic-cardiomyopathy
#17
Michele Covella, Ethan J Rowin, Nicholas S Hill, Ioana R Preston, Alberto Milan, Alexander R Opotowsky, Barry J Maron, Martin S Maron, Bradley A Maron
BACKGROUND: There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure. METHODS AND RESULTS: To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status...
April 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28390176/cucurbitacin-b-protects-against-pressure-overload-induced-cardiac-hypertrophy
#18
Yang Xiao, Zheng Yang, Qing-Qing Wu, Xiao-Han Jiang, Yuan Yuan, Wei Chang, Zhou Yan Bian, Jin Xiu Zhu, Qi-Zhu Tang
Lack of effective anti-cardiac hypertrophy drugs creates a major cause for the increasing prevalence of heart failure. In the present study, we determined the anti-hypertrophy and anti-fibrosis potential of a natural plant triterpenoid, Cucurbitacin B both in vitro and in vivo. Aortic banding (AB) was performed to induce cardiac hypertrophy. After 1 week of surgery, mice were receive cucurbitacin B treatment (Gavage, 0.2 mg/kg body weight/2 day). Afer 4 weeks of AB, cucurbitacin B demonstrated a strong anti- hypertrophy and anti-fibrosis ability as evidenced by decreased of heart weight, myocardial cell cross-sectional area and interstitial fibrosis, ameliorated of systolic and diastolic abnormalities, normalized in gene expression of hypertrophic and fibrotic markers, reserved microvascular density in pressure overload induced hypertrophic mice...
April 8, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28382463/micrornas-association-in-the-cardiac-hypertrophy-secondary-to-complex-congenital-heart-disease-in-children
#19
Ma C Sánchez-Gómez, K A García-Mejía, M Pérez-Díaz Conti, G Díaz-Rosas, I Palma-Lara, R Sánchez-Urbina, M Klünder-Klünder, J A Botello-Flores, N A Balderrábano-Saucedo, A Contreras-Ramos
Complex congenital heart disease (CHD) affects cardiac blood flow, generating a pressure overload in the compromised ventricles and provoking hypertrophy that over time will induce myocardial dysfunction and cause a potential risk of imminent death. Therefore, the early diagnosis of complex CHD is paramount during the first year of life, with surgical treatment of patients favoring survival. In the present study, we analyzed cardiac tissue and plasma of children with cardiac hypertrophy (CH) secondary to CHD for the expression of 11 miRNAs specific to CH in adults...
April 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28378431/left-ventricular-biomechanics-in-professional-football-players
#20
Thomas G von Lueder, Anders Hodt, Gard F Gjerdalen, Kjetil Steine
Chronic exercise induces adaptive changes of left ventricular (LV) ejection and filling capacities which may be detected by novel speckle-tracking echocardiography (STE) and tissue Doppler imaging (TDI)-based techniques. 103 consecutive male elite Norwegian soccer players and 46 age-matched healthy controls underwent echocardiography at rest. STE was used to assess LV torsional mechanics and LV systolic longitudinal strain (LS). Diastolic function was evaluated by trans-mitral blood flow, mitral annular velocities by TDI, and LV inflow propagation velocity by colour M-mode...
April 4, 2017: Scandinavian Journal of Medicine & Science in Sports
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