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Dilated cardiomyopathy

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https://www.readbyqxmd.com/read/28087564/right-ventricular-ejection-fraction-is-incremental-to-left-ventricular-ejection-fraction-for-the-prediction-of-future-arrhythmic-events-in-patients-with-systolic-dysfunction
#1
Yoko Mikami, Umjeet Jolly, Bobak Heydari, Mingkai Peng, Fahad Almehmadi, Mohammed Zahrani, Mahmoud Bokhari, John Stirrat, Carmen P Lydell, Andrew G Howarth, Raymond Yee, James A White
BACKGROUND: Left ventricular ejection fraction remains the primary risk stratification tool used in the selection of patients for implantable cardioverter defibrillator therapy. However, this solitary marker fails to identify a substantial portion of patients experiencing sudden cardiac arrest. In this study, we examined the incremental value of considering right ventricular ejection fraction for the prediction of future arrhythmic events in patients with systolic dysfunction using the gold standard of cardiovascular magnetic resonance...
January 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28087342/myeloid-leukemia-factor-1-is-a-novel-modulator-of-neonatal-rat-cardiomyocyte-proliferation
#2
Ashraf Yusuf Rangrez, Jost Pott, Annika Kluge, Robert Frauen, Katharina Stiebeling, Phillip Hoppe, Samuel Sossalla, Norbert Frey, Derk Frank
The present study focuses on the identification of the gene expression profile of neonatal rat cardiomyocytes (NRVCMs) after dynamic mechanical stretch through microarrays of RNA isolated from cells stretched for 2, 6 or 24h. In this analysis, myeloid leukemia factor-1 (MLF1) was found to be significantly downregulated during the course of stretch. We found that MLF1 is highly expressed in the heart, however, its cardiac function is unknown yet. In line with microarray data, MLF1 was profoundly downregulated in in vivo mouse models of cardiomyopathy, and also significantly reduced in the hearts of human patients with dilated cardiomyopathy...
January 10, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28080919/restrictive-cardiomyopathy-and-secondary-congestive-heart-failure-in-a-mcdowell-s-carpet-python-morelia-spilota-mcdowelli
#3
Lionel Schilliger, Valérie Chetboul, Cécile Damoiseaux, Alexandra Nicolier
Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations...
December 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28077817/prognostic-significance-of-non-dilated-left-ventricular-size-and-mitral-regurgitation-in-patients-with-dilated-phase-of-hypertrophic-cardiomyopathy
#4
Toru Kubo, Yuichi Baba, Takayoshi Hirota, Katsutoshi Tanioka, Naohito Yamasaki, Yoshinori L Doi, Hiroaki Kitaoka
Although a subtype of hypertrophic cardiomyopathy (HCM), dilated phase of HCM (D-HCM) characterized by left ventricular (LV) systolic dysfunction, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period. The degree of LV dilatation and functional mitral regurgitation (MR) are generally thought to be important predictors of poor prognosis in patients with LV systolic dysfunction. However, there is little information available on the relations among LV size, presence of significant MR, and prognosis in D-HCM patients...
January 11, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28074340/clinical-significance-of-cathepsin-l-and-cathepsin-b-in-dilated-cardiomyopathy
#5
Siddharth Mehra, Manish Kumar, Mansi Manchanda, Ratnakar Singh, Bhaskar Thakur, Neha Rani, Sudheer Arava, Rajiv Narang, Dharamvir Singh Arya, Shyam S Chauhan
Dysregulated expression of lysosomal cysteine cathepsins is associated with adverse cardiac remodeling, a characteristic of several cardiovascular diseases. However, the information regarding the role of cysteine cathepsin L (CTSL) and cathepsin B (CTSB) in dilated cardiomyopathy (DCM) is limited. The present study was aimed to investigate the expression of CTSL and CTSB in animal model of doxorubicin (doxo)-induced cardiomyopathy as well as in peripheral blood samples of DCM patients. Cardiac tissue sections from doxo-treated and control rats were used to study the expression of CTSL and CTSB by enzyme assay and immunohistochemistry (IHC)...
January 10, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28070695/barth-syndrome-connecting-cardiolipin-to-cardiomyopathy
#6
REVIEW
Nikita Ikon, Robert O Ryan
The Barth syndrome (BTHS) is caused by an inborn error of metabolism that manifests characteristic phenotypic features including altered mitochondrial membrane phospholipids, lactic acidosis, organic acid-uria, skeletal muscle weakness and cardiomyopathy. The underlying cause of BTHS has been definitively traced to mutations in the tafazzin (TAZ) gene locus on chromosome X. TAZ encodes a phospholipid transacylase that promotes cardiolipin acyl chain remodeling. Absence of tafazzin activity results in cardiolipin molecular species heterogeneity, increased levels of monolysocardiolipin and lower cardiolipin abundance...
January 9, 2017: Lipids
https://www.readbyqxmd.com/read/28070248/childhood-cardiomyopathies-a-study-in-tertiary-care-hospital-in-upper-egypt
#7
Mohamed Abd Elaal Bakeet, Montaser Mohamed Mohamed, Ahmed Ahmed Allam, Rania Gamal
INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital...
November 2016: Electronic Physician
https://www.readbyqxmd.com/read/28069793/decreased-wnt-%C3%AE-catenin-signalling-contributes-to-the-pathogenesis-of-dilated-cardiomyopathy-caused-by-mutations-in-the-lamin-a-c-gene
#8
Caroline Le Dour, Coline Macquart, Fusako Sera, Shunichi Homma, Gisele Bonne, John P Morrow, Howard J Worman, Antoine Muchir
Cardiomyopathy caused by lamin A/C gene (LMNA) mutations (hereafter referred as LMNA cardiomyopathy) is characterized by cardiac conduction abnormalities and left ventricular systolic dysfunction predisposing to heart failure. Previous cardiac transcriptional profiling of Lmna(H222P/H222P) mouse, a small animal model of LMNA cardiomyopathy, suggested decreased WNT/β-catenin signalling. We confirmed decreased WNT/β-catenin signalling in the hearts of these mice by demonstrating decreased β-catenin and WNT proteins...
January 9, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28069640/sequence-variation-in-ppp1r13l-results-in-a-novel-form-of-cardio-cutaneous-syndrome
#9
Tzipora C Falik-Zaccai, Yiftah Barsheshet, Hanna Mandel, Meital Segev, Avraham Lorber, Shachaf Gelberg, Limor Kalfon, Shani Ben Haroush, Adel Shalata, Liat Gelernter-Yaniv, Sarah Chaim, Dorith Raviv Shay, Morad Khayat, Michal Werbner, Inbar Levi, Yishay Shoval, Galit Tal, Stavit Shalev, Eli Reuveni, Emily Avitan-Hersh, Eugene Vlodavsky, Liat Appl-Sarid, Dorit Goldsher, Reuven Bergman, Zvi Segal, Ora Bitterman-Deutsch, Orly Avni
Dilated cardiomyopathy (DCM) is a life-threatening disorder whose genetic basis is heterogeneous and mostly unknown. Five Arab Christian infants, aged 4-30 months from four families, were diagnosed with DCM associated with mild skin, teeth, and hair abnormalities. All passed away before age 3. A homozygous sequence variation creating a premature stop codon at PPP1R13L encoding the iASPP protein was identified in three infants and in the mother of the other two. Patients' fibroblasts and PPP1R13L-knocked down human fibroblasts presented higher expression levels of pro-inflammatory cytokine genes in response to lipopolysaccharide, as well as Ppp1r13l-knocked down murine cardiomyocytes and hearts of Ppp1r13l-deficient mice...
January 9, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28069158/systolic-myocardial-volume-gain-in-dilated-hypertrophied-and-normal-heart-cmr-study
#10
Ł Mazurkiewicz, E Orłowska-Baranowska, J Petryka, M Śpiewak, M Gawor, B Miłosz-Wieczorek, K Werys, Ł A Małek, M Marczak, J Grzybowski
AIM: To investigate changes in myocardial tissue volume during the cardiac cycle to verify the hypothesis of non-compressibility of the myocardium in healthy individuals (HI) as well as in patients with hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and aortic stenosis (AS). MATERIALS AND METHODS: The study group included 30 HI, and patients with HCM (n=110), DCM (n=89), and AS (n=78). Left ventricular (LV) function, end-diastolic, and end-systolic volumes were calculated based on cardiac magnetic resonance imaging (CMR) for all participants...
January 6, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28068282/serological-and-molecular-biological-studies-of-parvovirus-b19-coxsackie-b-viruses-and-adenoviruses-as-potential-cardiotropic-viruses-in-bulgaria
#11
Stefka Kr Ivanova, Svetla G Angelova, Asya P Stoyanova, Irina L Georgieva, Lubomira K Nikolaeva-Glomb, Zafira G Mihneva, Neli St Korsun
BACKGROUND: Inflammatory diseases of the heart (myocarditis, pericarditis) are commonly caused by viruses. Among the human cardiotropic viruses, parvovirus B19, Coxsackie B viruses, and adenoviruses play a leading role. AIM: The aim of the present study was to determine the presumptive causative role of parvovirus B19, Coxsackie B viruses, and adenoviruses in the development of myocarditis, pericarditis and dilated cardiomyopathy by demonstrating the presence of specific antiviral antibodies or viral DNA in patients' serum samples...
December 1, 2016: Folia Medica
https://www.readbyqxmd.com/read/28067713/widespread-down-regulation-of-cardiac-mitochondrial-and-sarcomeric-genes-in-patients-with-sepsis
#12
Scot J Matkovich, Belal Al Khiami, Igor R Efimov, Sarah Evans, Justin Vader, Ashwin Jain, Bernard H Brownstein, Richard S Hotchkiss, Douglas L Mann
OBJECTIVES: The mechanism(s) for septic cardiomyopathy in humans is not known. To address this, we measured messenger RNA alterations in hearts from patients who died from systemic sepsis, in comparison to changed messenger RNA expression in nonfailing and failing human hearts. DESIGN: Identification of genes with altered abundance in septic cardiomyopathy, ischemic heart disease, or dilated cardiomyopathy, in comparison to nonfailing hearts. SETTING: ICUs at Barnes-Jewish Hospital, St...
January 6, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28065693/inhibition-of-mir-208b-improves-cardiac-function-in-titin-based-dilated-cardiomyopathy
#13
Qifeng Zhou, Sonja Schötterl, Daniel Backes, Eva Brunner, Julia Kelley Hahn, Elena Ionesi, Parwez Aidery, Carsten Sticht, Siegfried Labeit, Reinhard Kandolf, Meinrad Gawaz, Michael Gramlich
BACKGROUND: Dilated cardiomyopathy (DCM) is the result of maladaptive cardiac remodeling, which involves microRNA regulation. In turn, microRNAs can contribute to the remodeling process by post-transcriptional modulation of gene expression networks. The exact role of microRNAs in the pathogenesis of DCM is largely unknown. Here, we used an inducible DCM mouse model that carries a human truncation mutation in the sarcomeric protein titin to dissect microRNA pathways in DCM development...
December 28, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28062634/dilated-cardiomyopathy-in-a-2-year-old-infant
#14
Roy W A Peake, Kevin P Daly
No abstract text is available yet for this article.
January 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28056816/optimization-of-pressure-settings-during-adaptive-servo-ventilation-support-using-real-time-heart-rate-variability-assessment-initial-case-report
#15
Teruhiko Imamura, Daisuke Nitta, Koichiro Kinugawa
BACKGROUND: Adaptive servo-ventilation (ASV) therapy is a recent non-invasive positive pressure ventilation therapy that was developed for patients with heart failure (HF) refractory to optimal medical therapy. However, it is likely that ASV therapy at relatively higher pressure setting worsens some of the patients' prognosis compared with optimal medical therapy. Therefore, identification of optimal pressure settings of ASV therapy is warranted. CASE PRESENTATION: We present the case of a 42-year-old male with HF, which was caused by dilated cardiomyopathy, who was admitted to our institution for evaluating his eligibility for heart transplantation...
January 5, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28053874/long-term-outcome-of-isobutyryl-coa-dehydrogenase-deficiency-diagnosed-following-an-episode-of-ketotic-hypoglycaemia
#16
S Santra, A Macdonald, M A Preece, R K Olsen, B S Andresen
Isobutyryl-CoA Dehydrogenase Deficiency (IBDD) is an inherited disorder of valine metabolism caused by mutations in ACAD8. Most reported patients have been diagnosed through newborn screening programmes due to elevated C4-carnitine levels and appear clinically asymptomatic. One reported non-screened patient had dilated cardiomyopathy and anaemia at the age of two years. We report a 13 month old girl diagnosed with IBDD after developing hypoglycaemic encephalopathy (blood glucose 1.9 mmol/l) during an episode of rotavirus-induced gastroenteritis...
March 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28045975/titin-truncating-variants-in-dilated-cardiomyopathy-prevalence-and-genotype-phenotype-correlations
#17
Maria Franaszczyk, Przemyslaw Chmielewski, Grazyna Truszkowska, Piotr Stawinski, Ewa Michalak, Malgorzata Rydzanicz, Malgorzata Sobieszczanska-Malek, Agnieszka Pollak, Justyna Szczygieł, Joanna Kosinska, Adam Parulski, Tomasz Stoklosa, Agnieszka Tarnowska, Marcin M Machnicki, Bogna Foss-Nieradko, Malgorzata Szperl, Agnieszka Sioma, Mariusz Kusmierczyk, Jacek Grzybowski, Tomasz Zielinski, Rafal Ploski, Zofia T Bilinska
TTN gene truncating variants are common in dilated cardiomyopathy (DCM), although data on their clinical significance is still limited. We sought to examine the frequency of truncating variants in TTN in patients with DCM, including familial DCM (FDCM), and to look for genotype-phenotype correlations. Clinical cardiovascular data, family histories and blood samples were collected from 72 DCM probands, mean age of 34 years, 45.8% FDCM. DNA samples were examined by next generation sequencing (NGS) with a focus on the TTN gene...
2017: PloS One
https://www.readbyqxmd.com/read/28045725/utilization-of-stand-by-extracorporeal-membrane-oxygenation-in-a-high-risk-parturient-with-methamphetamine-associated-cardiomyopathy-undergoing-dilation-and-evacuation-a-case-report
#18
Cesar Padilla, Antonio Hernandez Conte, Danny Ramzy, Lorraine Lubin, Troy LaBounty, Judith H Chung, Ying Zeng
Parturients may present with evidence of acute heart failure or respiratory distress during the peripartum period. This case report documents utilization of "stand-by" extracorporeal membrane oxygenation (ECMO) for a 40-year-old woman with a history of severe left ventricular dysfunction who presented for elective dilation and evacuation of triplets at 20 weeks' gestation. The patient's medical history was significant for hypertension, diabetes mellitus, methamphetamine use (acute/chronic), and cardiac-respiratory arrest before her previous emergent cesarean delivery...
December 30, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/28045696/sudden-death-in-a-male-infant-due-to-histiocytoid-cardiomyopathy-an-autopsy-case-and-review-of-the-literature
#19
Hanbing Xie, Xueqin Chen, Ni Chen, Qiao Zhou
Histiocytoid cardiomyopathy (HC) is a very rare cardiac disorder that mainly affects female infants younger than 2 years. It may manifest as ventricular tachycardia or dilated cardiomyopathy and frequently causes sudden death. The most common grossly change is multifocal uneven thickening of the endocardium, presenting a yellowish color, with some area forming nodular appearance, and histologically featured by scattered clusters of histiocytoid myocytes under the endocardium. Here, we present a 19-month-old male infant who died of heart failure, and an autopsy was performed and confirmed the diagnosis of HC...
December 30, 2016: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/28043679/presence-of-implantable-cardioverter-defibrillators-and-wait-list-mortality-of-patients-supported-with-left-ventricular-assist-devices-as-bridge-to-heart-transplantation
#20
Ahmad Younes, Sadeer G Al-Kindi, Wissam Alajaji, Judith A Mackall, Guilherme H Oliveira
BACKGROUND: The role of implantable cardioverter defibrillator (ICD) in reducing mortality in patients with left ventricular assisted devices (LVADs) listed for heart transplant remains unclear. We therefore, sought to interrogate whether ICDs are associated with reduced mortality in patients with LVADs listed for heart transplantation. METHODS: We searched the United Network for Organ Sharing (UNOS) Registry for LVAD patients (age≥18years) with dilated cardiomyopathies listed for heart transplantation (2008-2015)...
December 28, 2016: International Journal of Cardiology
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