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https://www.readbyqxmd.com/read/28431892/a-humanized-hla-dr4-mouse-model-for-autoimmune-myocarditis
#1
M Emrah Şelli, Anita C Thomas, David C Wraith, Andrew C Newby
Myocarditis, the principal cause of dilated cardiomyopathy and heart failure in young adults, is associated with autoimmunity to human cardiac α-myosin (hCAM) and the DR4 allele of human major histocompatibility II (MHCII). We developed an hCAM-induced myocarditis model in human HLA-DR4 transgenic mice that lack all mouse MHCII genes, demonstrating that immunization for 3weeks significantly increased splenic T-cell proliferative responses and titres of IgG1 and IgG2c antibodies, abolished weight gain, provoked cardiac inflammation and significantly impaired cardiac output and fractional shortening, by echocardiography, compared to adjuvant-injected mice...
April 18, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28431057/quantitative-analysis-of-pkp2-and-neighbouring-genes-in-a-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-caused-by-heterozygous-pkp2-deletion
#2
Keiko Sonoda, Seiko Ohno, Sou Otuki, Koichi Kato, Nobue Yagihara, Hiroshi Watanabe, Takeru Makiyama, Tohru Minamino, Minoru Horie
Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease mainly caused by desmosome gene mutations. The genetic culprit, however, remains elusive in ∼50% of ARVC patients. One of the reasons for missing genetic abnormalities is the difficulty in detecting large deletions/duplications, which are called as copy number variation (CNV) by the Sanger sequencing method. This study aimed to identify CNVs in PKP2 and a part of other desmosome genes in ARVC patients. Methods and Results: The study cohort consisted of 71 ARVC probands who were diagnosed as definite or borderline cases based on 2010 Task Force Criteria...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28431056/long-term-outcome-of-transvenous-pacemaker-implantation-in-infants-a-retrospective-cohort-study
#3
Laura M Vos, Janneke A E Kammeraad, Matthias W Freund, Andreas C Blank, Johannes M P J Breur
Aim: Evaluation of long-term outcome of transvenous pacemaker (PM) implantation in infants. Methods and Results: A retrospective analysis of all transvenous PM implantations in infants <10 kg between September 1997 and October 2001 was made. Indications for PM implantation, age at implantation, and determinants of long-term outcome including cardiac function, PM function, and PM (system) complications were noted. Seven patients underwent transvenous VVI(R) PM implantation...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28430627/the-long-non-coding-rna-h19-promotes-cardiomyocyte-apoptosis-in-dilated-cardiomyopathy
#4
Yanlin Zhang, Mengyao Zhang, Weiting Xu, Jianchang Chen, Xiang Zhou
In the previous study, we generated a rat model of dilated cardiomyopathy (DCM) induced by adriamycin and found that the expression of lncRNA H19 was significantly upregulated in myocardial tissue. The present study was aimed to investigate the potential role of H19 in the pathogenesis of adriamycin-induced DCM. H19 knockdown in the myocardium of DCM rats attenuated cardiomyocyte apoptosis and improved left ventricular structure and function. Adriamycin treatment was associated with elevated H19 and miR-675 expression and increased apoptosis in neonatal cardiomyocytes...
February 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/28429347/sumoylation-in-cardiac-disorders-a-review
#5
X-C Li, Y Zeng, R-R Sun, M Liu, S Chen, P-Y Zhang
SUMOylation regulates diverse cellular processes including transcription, cell cycle, protein stability, and apoptosis. A recent research has now revealed the role of SUMO1 in cardiac disorders. Studies have evidenced that failing heart induces SUMO2/3 conjugation. Moreover, increased SUMO2/3- dependent modification has been observed to result in congestive heart disease such as cardiac hypertrophy by promoting cardiac cell death. Also, few recent studies have confirmed the role of SUMOylation in cardiac protein degradation...
April 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28427148/in-depth-proteomic-profiling-of-left-ventricular-tissues-in-human-end-stage-dilated-cardiomyopathy
#6
Shanshan Liu, Yan Xia, Xiaohui Liu, Yi Wang, Zhangwei Chen, Juanjuan Xie, Juying Qian, Huali Shen, Pengyuan Yang
Dilated cardiomyopathy (DCM) is caused by reduced left ventricular (LV) myocardial function, which is one of the most common causes of heart failure (HF). We performed iTRAQ-coupled 2D-LC-MS/MS to profile the cardiac proteome of LV tissues from healthy controls and patients with end-stage DCM. We identified 4263 proteins, of which 125 were differentially expressed in DCM tissues compared to LV controls. The majority of these were membrane proteins related to cellular junctions and neuronal metabolism. In addition, these proteins were involved in membrane organization, mitochondrial organization, translation, protein transport, and cell death process...
February 25, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423146/recovery-in-patients-with-dilated-cardiomyopathy-with-loss-of-function-mutations-in-the-titin-gene
#7
Kevin Luk, Abeer Bakhsh, Nadia Giannetti, Eleanor Elstein, Mark Lathrop, George Thanassoulis, James C Engert
No abstract text is available yet for this article.
April 19, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28423127/evaluation-of-anomalous-coronary-arteries-from-the-pulmonary-artery
#8
Alper Guzeltas, Erkut Ozturk, Ibrahim Cansaran Tanidir, Taner Kasar, Sertac Haydin
Objective: This study evaluated clinical and diagnostic findings, treatment methods, and follow-up of cases of anomalous coronary arteries from the pulmonary artery. Methods: The study included all cases diagnosed with anomalous coronary arteries from the pulmonary artery between January 2012 and January 2016. Data from patients' demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, intensive care unit stay, and follow-up were evaluated...
January 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28420549/predictors-of-left-ventricular-functional-recovery-and-their-impact-on-clinical-outcomes-in-patients-with-newly-diagnosed-dilated-cardiomyopathy-and-heart-failure
#9
Jae Yeong Cho, Kye Hun Kim, Ji Eun Song, Ji Eun Kim, Hyukjin Park, Hyun Ju Yoon, Nam Sik Yoon, Young Joon Hong, Hyung Wook Park, Ju Han Kim, Youngkeun Ahn, Myung Ho Jeong, Jeong Gwan Cho, Jong Chun Park
BACKGROUND: To identify the predictors of left ventricular functional recovery (LVFR) and its impacts on clinical outcomes in acute heart failure (AHF) patients with newly diagnosed dilated cardiomyopathy (DCM). METHODS: A total of 175 consecutive patients with newly diagnosed DCM and AHF were divided into two groups according to LVFR on FU echocardiography; the recovered group (n=54, 54.3±18.5years, 31 males) vs. the non-recovered group (n=121, 60.5±15.1years, 79 males)...
March 20, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28418113/autochthonous-chagas-disease-in-the-southern-united-states-a-case-report-of-suspected-residential-and-military-exposures
#10
N Harris, L Woc-Colburn, S M Gunter, R Gorchakov, K O Murray, S Rossmann, M N Garcia
Chagas disease is a parasitic infection that can result in a progressive dilated cardiomyopathy. Here, we present the epidemiologic details of a suspected locally acquired transmission case originating from the southern United States. This is the first published report of Chagas disease in a young, healthy United States veteran with repeat triatomine exposures in Arizona. Military personnel and Arizona residents should be aware of their Chagas disease transmission risks.
April 18, 2017: Zoonoses and Public Health
https://www.readbyqxmd.com/read/28416588/association-between-mutation-status-and-left-ventricular-reverse-remodelling-in-dilated-cardiomyopathy
#11
Matteo Dal Ferro, Davide Stolfo, Alessandro Altinier, Marta Gigli, Martina Perrieri, Federica Ramani, Giulia Barbati, Alberto Pivetta, Francesca Brun, Lorenzo Monserrat, Mauro Giacca, Luisa Mestroni, Marco Merlo, Gianfranco Sinagra
OBJECTIVE: To explore the genetic landscape of a well selected dilated cardiomyopathy (DCM) cohort, assessing the possible relation between different genotypes and left ventricular reverse remodelling (LVRR). METHODS: A cohort of 152 patients with DCM from the Heart Muscle Disease Registry of Trieste has been studied by next-generation sequencing (NGS). Patients were grouped into different 'gene-clusters' with functionally homogeneous genetic backgrounds. LVRR was defined by left ventricular ejection fraction normalisation or increase ≥10% associated with normalisation in indexed left ventricular end-diastolic diameter or relative decrease ≥10% at 24 months follow-up...
April 17, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28414672/a-strong-and-fortuitous-case-of-dyspnea
#12
R Hammer, M Sciaudone
CASE: A 48 year-old man with no past medical history was sent to our emergency department (ED); from a primary care clinic for hypertensive urgency of 200/130. The man reported an intermittent non-productive cough of approximately one year's duration and worsening dyspnea on exertion and orthopnea over the last month with lower extremity swelling. Of note, he emigrated from Honduras twenty years ago. Blood pressure normalized with administration of Lasix in the ED. Physical exam revealed rales in lung bases bilaterally, jugular venous distension, lower extremity pitting edema with serpiginous patches of erythema and excoriation, and a cardiac gallop...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
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
#13
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/28408455/cell-therapy-trials-in-congenital-heart-disease
#14
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28407410/variable-clinical-course-of-identical-twin-neonates-with-alstr%C3%A3-m-syndrome-presenting-coincidentally-with-dilated-cardiomyopathy
#15
Seth A Hollander, Norah Alsaleh, Maura Ruzhnikov, Kristen Jensen, David N Rosenthal, David A Stevenson, Melanie Manning
Alström Syndrome (AS) is a rare autosomal recessive disorder caused by mutations in the ALMS1 gene. We report monozygotic twin infants who presented concurrently with symptoms of congestive heart failure (CHF) due to dilated cardiomyopathy (DCM). Following their initial presentation, one twin improved both echocardiographically and functionally while the other twin showed a progressive decline in ventricular function and worsening CHF symptoms requiring multiple hospitalizations and augmentation of heart failure therapy...
April 13, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28402404/long-term-outcome-of-surgical-cryoablation-for-refractory-ventricular-tachycardia-in-patients-with-non-ischemic-cardiomyopathy
#16
Jackson J Liang, Brian P Betensky, Daniele Muser, Erica S Zado, Elad Anter, Nimesh D Desai, David J Callans, Rajat Deo, David S Frankel, Mathew D Hutchinson, David Lin, Michael P Riley, Robert D Schaller, Gregory E Supple, Pasquale Santangeli, Michael A Acker, Joseph E Bavaria, Wilson Y Szeto, Prashanth Vallabhajosyula, Francis E Marchlinski, Sanjay Dixit
Aims: Limited data exist on the long-term outcome of patients (pts) with non-ischemic cardiomyopathy (NICM) and ventricular tachycardia (VT) refractory to conventional therapies undergoing surgical ablation (SA). We aimed to investigate the long-term survival and VT recurrence in NICM pts with VT refractory to radiofrequency catheter ablation (RFCA) who underwent SA. Methods and results: Consecutive pts with NICM and VT refractory to RFCA who underwent SA were included...
April 10, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28401079/mutation-in-%C3%AE-sg-gene-in-familial-dilated-cardiomyopathy
#17
Marzieh Asadi, Roger Foo, Ahmad Reza Salehi, Rasoul Salehi, Mohammad Reza Samienasab
BACKGROUND: Mutations in different genes including dystrophin-associated glycoprotein complex caused familial dilated cardiomyopathy which is a genetically heterogeneous disease. The δ-SG gene contains nine exons spanning a 433-kb region of genomic DNA. It encodes a 35-kDa, singlepass, and type II transmembrane glycoprotein. MATERIALS AND METHODS: In this study for the first time in Iran we screened 6 patients of a large family that they had positive family history of MI or sudden death by next generation sequencing method...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28400684/early-prediction-of-3-month-survival-of-patients-in-refractory-cardiogenic-shock-and-cardiac-arrest-on-extracorporeal-life-support
#18
Clément Delmas, Jean-Marie Conil, Simon Sztajnic, Bernard Georges, Caroline Biendel, Camille Dambrin, Michel Galinier, Vincent Minville, Olivier Fourcade, Stein Silva, Bertrand Marcheix
BACKGROUND: Extracorporeal life support (ECLS) holds the promise of significant improvement of the survival of patient in refractory cardiogenic shock (CS) or cardiac arrest (CA). Nevertheless, it remains to be shown to which extent these highly invasive supportive techniques could improve long-term patient's outcome. METHODS: The outcomes of 82 adult ECLS patients at our institution between January 2012 and December 2013 were retrospectively analyzed. RESULTS: Patients were essentially men (64...
March 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28398556/prognosis-associated-with-redo-cardiac-resynchronization-therapy-following-complete-device-and-lead-extraction-due-to-device-related-infection
#19
Martin Seifert, Viviane Moeller, Anita Arya, Thomas Schau, Frank Hoelschermann, Christian Butter
Aims: An increase in the number of cardiac resynchronization therapy (CRT) device implantations worldwide has led to a consequent increase in the number of infections associated with the device, making extraction of the CRT device inevitable. Redo CRT implantation after treatment and recovery following device extraction is challenging. This study aimed to evaluate the success rate, complications, and long-term prognosis of redo CRT implantation, including the rates of subclavian, cava, and coronary sinus (CS) vein thrombosis as well as re-infection...
April 6, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28398466/novel-nesprin-1-mutations-associated-with-dilated-cardiomyopathy-cause-nuclear-envelope-disruption-and-defects-in-myogenesis
#20
Can Zhou, Chen Li, Bin Zhou, Huaqin Sun, Victoria Koullourou, Ian Holt, Megan J Puckelwartz, Derek T Warren, Robert Hayward, Ziyuan Lin, Lin Zhang, Glenn E Morris, Elizabeth M McNally, Sue Shackleton, Li Rao, Catherine M Shanahan, Qiuping Zhang
Nesprins-1 and -2 are highly expressed in skeletal and cardiac muscle and together with SUN (Sad1p/UNC84)-domain containing proteins and lamin A/C form the LInker of Nucleoskeleton-and-Cytoskeleton (LINC) bridging complex at the nuclear envelope (NE). Mutations in nesprin-1/2 have previously been found in patients with autosomal dominant Emery-Dreifuss muscular dystrophy (EDMD) as well as dilated cardiomyopathy (DCM). In this study, three novel rare variants (R8272Q, S8381C and N8406K) in the C-terminus of the SYNE1 gene (nesprin-1) were identified in seven DCM patients by mutation screening...
April 7, 2017: Human Molecular Genetics
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