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idiopathic dilated cardiomyopathy

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https://www.readbyqxmd.com/read/28212578/interleukin-1%C3%AE-levels-predict-long-term-mortality-and-need-for-heart-transplantation-in-ambulatory-patients-affected-by-idiopathic-dilated-cardiomyopathy
#1
Aneta Aleksova, Antonio Paolo Beltrami, Cosimo Carriere, Giulia Barbati, Pierluigi Lesizza, Martina Perrieri-Montanino, Miriam Isola, Piero Gentile, Elisabetta Salvioni, Tarcisio Not, Piergiuseppe Agostoni, Gianfranco Sinagra
AIMS: The prognostic stratification of patients with Idiopathic Dilated Cardiomyopathy (iDCM) is a difficult task. Here, we assessed the additive value of the evaluation of biomarkers of inflammasome activation and systemic inflammation for the long-term risk stratification of iDCM patients. METHODS AND RESULTS: We studied 156 ambulatory iDCM patients (mean age 58 years, 77% men, 79% in NYHA class 1-2, median Left Ventricular Ejection Fraction (LVEF) 35%, mean sodium 139 mEq/L, median BNP 189 pg/mL, median IL-1 beta (IL-1β) 1...
February 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28193717/differences-in-presentation-and-outcomes-between-children-with-familial-dilated-cardiomyopathy-and-children-with-idiopathic-dilated-cardiomyopathy-a-report-from-the-pediatric-cardiomyopathy-registry-study-group
#2
Paolo Rusconi, James D Wilkinson, Lynn A Sleeper, Minmin Lu, Gerald F Cox, Jeffrey A Towbin, Steven D Colan, Steven A Webber, Charles E Canter, Stephanie M Ware, Daphne T Hsu, Wendy K Chung, John L Jefferies, Christina Cordero, Steven E Lipshultz
BACKGROUND: Research comparing the survival of children with familial dilated cardiomyopathy (FDCM) to that of children with idiopathic dilated cardiomyopathy (IDCM) has produced conflicting results. METHODS AND RESULTS: We analyzed data from children with FDCM or IDCM using the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry. Compared to children with IDCM (n=647), children with FDCM (n=223) were older (mean 6.2 versus 4.5 years, P<0...
February 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28158878/psoriasis-and-cardiomyopathy-a-review-of-the-literature
#3
Taimoor Hashim, Amier Ahmad, Ayesha Chaudry, Rami Khouzam
Psoriasis is an idiopathic chronic immune-mediated skin condition in which the body's immune system undergoes several derangements, including increased antigen presentation by T cells and increased T-helper cell type 1 cytokines, resulting in skin lesions as well as arthritis. Despite that cardiovascular involvement in psoriasis is common and reported in up to 47% of cases, this association is not well recognized by physicians, dermatologists, and cardiologists. Psoriasis is considered the most prevalent autoimmune disease in the United States and affects approximately 7...
February 2017: Southern Medical Journal
https://www.readbyqxmd.com/read/28149663/left-ventricular-systolic-dysfunction-in-two-patients-with-ankylosing-spondylitis-what-is-the-role-of-corticosteroids
#4
Ahmad Amin, Mitra Chitsazan, Hossein Navid
Ankylosing spondylitis (AS) is a chronic inflammatory condition that most commonly affects the axial skeleton. The most common cardiac manifestation in patients with AS is the aortic root and valve disease, followed by conduction and rhythm abnormalities, decreased coronary flow reserve, myocardial infarction, and diastolic dysfunction. However, the presence of systolic dysfunction has been less described in patients with AS. Herein we present two cases of idiopathic dilated cardiomyopathy in patients with AS...
December 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28138556/myocardial-micrornas-associated-with-reverse-remodeling-in-human-heart-failure
#5
Carmen C Sucharov, David P Kao, J David Port, Anis Karimpour-Fard, Robert A Quaife, Wayne Minobe, Karin Nunley, Brian D Lowes, Edward M Gilbert, Michael R Bristow
BACKGROUND: In dilated cardiomyopathies (DCMs) changes in expression of protein-coding genes are associated with reverse remodeling, and these changes can be regulated by microRNAs (miRs). We tested the general hypothesis that dynamic changes in myocardial miR expression are predictive of β-blocker-associated reverse remodeling. METHODS: Forty-three idiopathic DCM patients (mean left ventricular ejection fraction 0.24 ± 0.09) were treated with β-blockers. Serial ventriculography and endomyocardial biopsies were performed at baseline, and after 3 and 12 months of treatment...
January 26, 2017: JCI Insight
https://www.readbyqxmd.com/read/28133686/neutrophil-to-lymphocyte-ratio-predicts-appropriate-therapy-in-idiopathic-dilated-cardiomyopathy-patients-with-primary-prevention-implantable-cardioverter-defibrillator
#6
Fatih M Uçar, Burak Açar
OBJECTIVES: To investigate whether an inflammatory marker of neutrophil to lymphocyte ratio (NLR) predicts appropriate implantable cardioverter defibrillator (ICD) therapy (shock or anti tachycardia pacing) in idiopathic dilated cardiomyopathy (IDC) patients. METHODS: We retrospectively examined IDC patients (mean age: 58.3 ± 11.8 years, 81.5% male) with ICD who admitted to outpatient clinic for pacemaker control at 2 tertiary care hospitals in Ankara and Edirne, Turkey from January 2013-2015...
February 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28130335/development-of-dilated-cardiomyopathy-and-impaired-calcium-homeostasis-with-cardiac-specific-deletion-of-esrr%C3%AE
#7
Glenn C Rowe, Angeliki Asimaki, Evan L Graham, Kimberly D Martin, Kenneth B Margulies, Saumya Das, Jeffrey E Saffitz, Zoltan Arany
Mechanisms underlying the development of Idiopathic Dilated Cardiomyopathy (DCM) remain poorly understood. Using transcription factor expression profiling, we identified estrogen-related receptor beta (ESRRβ), a member of the nuclear receptor family of transcription factors, as highly expressed in murine hearts and other highly oxidative striated muscle beds. Mice bearing cardiac-specific deletion of ESRRβ (MHC-ERRB KO) develop dilated cardiomyopathy and sudden death at approximately 10 months of age. Isolated adult cardiomyocytes from the MHC-ERRB KO mice showed an increase in calcium sensitivity and impaired cardiomyocyte contractility, which preceded echocardiographic cardiac remodeling and dysfunction by several months...
January 27, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28123761/clinical-disease-presentation-and-ecg-characteristics-of-lmna-mutation-carriers
#8
Laura Ollila, Kjell Nikus, Miia Holmström, Mikko Jalanko, Raija Jurkko, Maija Kaartinen, Juha Koskenvuo, Johanna Kuusisto, Satu Kärkkäinen, Eeva Palojoki, Eeva Reissell, Päivi Piirilä, Tiina Heliö
OBJECTIVE: Mutations in the LMNA gene encoding lamins A and C of the nuclear lamina are a frequent cause of cardiomyopathy accounting for 5-8% of familial dilated cardiomyopathy (DCM). Our aim was to study disease onset, presentation and progression among LMNA mutation carriers. METHODS: Clinical follow-up data from 27 LMNA mutation carriers and 78 patients with idiopathic DCM without an LMNA mutation were collected. In addition, ECG data were collected and analysed systematically from 20 healthy controls...
2017: Open Heart
https://www.readbyqxmd.com/read/28120210/renin-angiotensin-system-gene-polymorphisms-as-potential-modifiers-of-hypertrophic-and-dilated-cardiomyopathy-phenotypes
#9
Bindu Rani, Amit Kumar, Ajay Bahl, Rajni Sharma, Rishikesh Prasad, Madhu Khullar
The renin-angiotensin (RAS) pathway has an important role in the etiology of heart failure and given the importance of RAS as a therapeutic target in various cardiomyopathies, genetic polymorphisms in the RAS genes may modulate the risk and severity of disease in cardiomyopathy patients. In the present study, we examined the association of RAS pathway gene polymorphisms, angiotensin converting enzyme (ACE), angiotensinogen (AGT), and angiotensin receptor type 1 (AGTR1) with risk and disease severity in Asian Indian idiopathic cardiomyopathy patients...
March 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28099117/casz1-loss-of-function-mutation-contributes-to-familial-dilated-cardiomyopathy
#10
Xing-Biao Qiu, Xin-Kai Qu, Ruo-Gu Li, Hua Liu, Ying-Jia Xu, Min Zhang, Hong-Yu Shi, Xu-Min Hou, Xu Liu, Fang Yuan, Yu-Min Sun, Jun Wang, Ri-Tai Huang, Song Xue, Yi-Qing Yang
BACKGROUND: The zinc finger transcription factor CASZ1 plays a key role in cardiac development and postnatal adaptation, and in mice, deletion of the CASZ1 gene leads to dilated cardiomyopathy (DCM). However, in humans whether genetically defective CASZ1 contributes to DCM remains unclear. METHODS: The coding exons and splicing junction sites of the CASZ1 gene were sequenced in 138 unrelated patients with idiopathic DCM. The available family members of the index patient harboring an identified CASZ1 mutation and 200 unrelated, ethnically matched healthy individuals used as controls were genotyped for CASZ1...
January 18, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28098235/molecular-characterization-of-pediatric-restrictive-cardiomyopathy-from-integrative-genomics
#11
Tara N Rindler, Robert B Hinton, Nathan Salomonis, Stephanie M Ware
Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28097671/junctional-ectopic-tachycardia-localization-and-procedural-approach-using-cryoablation
#12
Alyson R Pierick, Ian H Law, Jennifer R Muldonado, Nicholas H VON Bergen
BACKGROUND: Idiopathic junctional ectopic tachycardia (JET) may still be difficult to control with antiarrhythmic therapy. Transcatheter ablation can be challenging and may be associated with a high risk of unintended AV block. The objective of this manuscript is to report the procedural technique, the location of the successful ablation and the procedural characteristics while utilizing 3D mapping for cryoablation of JET. METHODS: A retrospective analysis was performed on all patients who had undergone cryothermal ablation for the treatment of JET at a single center...
January 18, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28035124/your-epoch-is-not-for-trying-it-s-for-living-and-for-dying
#13
Leonid P Churilov
The article is devoted to biographies of three Russian physicians of the Silver Age (a period in the History of Russian culture between 1890 and 1917). They made early, significant and internationally recognized contribution into medical science and became eponymous, although social disasters of the twentieth century caused deep impact on their subsequent lives and careers, so their role was shadowed from global medical community. The article analyzes biographies and academic achievements of A-F.K. Siewert (aka: Zivert, Ziwert, von Siewert) (1872-1922), known for first description of the hereditary dyskinesia of cilia (as a triad of: situs inversus of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis); S...
December 2016: Psychiatria Danubina
https://www.readbyqxmd.com/read/28013418/left-ventricular-reverse-remodeling-in-dilated-cardiomyopathy-maintained-subclinical-myocardial-systolic-and-diastolic-dysfunction
#14
Sandra Amorim, João Rodrigues, Manuel Campelo, Brenda Moura, Elisabete Martins, Filipe Macedo, J Silva-Cardoso, M Júlia Maciel
In idiopathic dilated cardiomyopathy (DCM), myocardial deformational parameters and their relationships remain incompletely characterized. We measured those parameters in patients with DCM, during left ventricular reverse remodeling (LVRR). Prospective study of 50 DCM patients (in sinus rhythm), with left ventricular ejection fraction (EF) <40%. LVRR was defined as an increase of ten units of EF and decrease of diastolic left ventricular diameter (LVDD) in the absence of resynchronization therapy. Performed morphological analysis, myocardial performance quantification (LV and RV Tei indexes) and LV averaged peak systolic longitudinal strain (SSR long) and circumferential strain (SSR circ)...
December 24, 2016: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28004791/free-triiodothyronine-level-correlates-with-myocardial-injury-and-prognosis-in-idiopathic-dilated-cardiomyopathy-evidence-from-cardiac-mri-and-spect-pet-imaging
#15
Wenyao Wang, Haixia Guan, Wei Fang, Kuo Zhang, A Martin Gerdes, Giorgio Iervasi, Yi-Da Tang
Thyroid dysfunction is associated with poor prognosis in heart failure, but theories of mechanisms are mainly based on animal experiments, not on human level. We aimed to explore the relation between thyroid function and myocardial injuries in idiopathic dilated cardiomyopathy (IDCM) using cardiac magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT) and positron emission tomography (PET). Myocardial fibrosis was detected by late gadolinium enhancement (LGE) MRI, and myocardial perfusion/metabolism was evaluated by (99m)Tc-MIBI SPECT /(18)F-FDG PET imaging...
December 22, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27988039/comparison-of-patient-characteristics-and-course-of-hypertensive-hypokinetic-cardiomyopathy-versus-idiopathic-dilated-cardiomyopathy
#16
Marco Bobbo, Bruno Pinamonti, Marco Merlo, Davide Stolfo, Annamaria Iorio, Federica Ramani, Giulia Barbati, Cosimo Carriere, Laura Massa, Stefano Poli, Sara Scapol, Marta Gigli, Andrea Di Lenarda, Gianfranco Sinagra
Hypertensive hypokinetic cardiomyopathy (HHC) is defined by left ventricular (LV) systolic dysfunction with a history of systemic hypertension as the only possible cause. Although commonly encountered in clinical practice, its characterization and differences with true idiopathic dilated cardiomyopathy (IDC) are lacking. The aim of this study was to characterize the clinical instrumental features and the natural history of HHC. We analyzed the data of 4,191 patients referred to our center for newly diagnosed LV systolic dysfunction from 2005 to 2010...
February 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27965028/two-de-novo-mutations-in-an-autistic-child-who-had-previously-undergone-transplantation-for-dilated-cardiomyopathy-the-importance-of-keeping-an-open-mind
#17
Umair Sajid, Bob Argiropoulos, Xing-Chang Wei, Jillian S Parboosingh, Ryan E Lamont, Aneal Khan, Steven C Greenway
We report the finding of 2 de novo mutations in an 8-year-old boy with developmental delay and autism who underwent heart transplantation at 1 year of age for idiopathic dilated cardiomyopathy. We identified a de novo microdeletion at chromosome 2p16.3 involving the neurexin-1 (NRXN1) gene and a de novo pathologic variant (Pro838Leu) in the myosin heavy chain 7 (MYH7) gene. This case emphasizes the importance of comprehensive genetic evaluation in patients with cardiomyopathy, particularly if they have extracardiac abnormalities, and the necessity of interpreting variants with attention to the phenotype...
September 26, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27890770/fibrosis-and-fibrotic-gene-expression-in-pediatric-and-adult-patients-with-idiopathic-dilated-cardiomyopathy
#18
Kathleen C Woulfe, Austine K Siomos, Hieu Nguyen, Megan SooHoo, Csaba Galambos, Brian L Stauffer, Carmen Sucharov, Shelley Miyamoto
BACKGROUND: Although fibrosis seems to be prognostic for adverse outcomes in adults with idiopathic dilated cardiomyopathy (IDC), little is known about the prevalence and development of fibrosis in pediatric IDC hearts. We hypothesized that there is less activation of fibrosis at a molecular level in pediatric IDC hearts than in failing adult hearts. METHODS AND RESULTS: Pediatric hearts were analyzed histologically to determine the prevalence of fibrosis. Left ventricular tissue from adult and pediatric IDC hearts and adult and pediatric nonfailing (NF) hearts were subjected to quantitative reverse-transcription polymerase chain reaction to study the expression of important mRNAs that affect fibrosis...
November 24, 2016: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/27889554/familial-dilated-cardiomyopathy-a-multidisciplinary-entity-from-basic-screening-to-novel-circulating-biomarkers
#19
REVIEW
D de Gonzalo-Calvo, M Quezada, O Campuzano, A Perez-Serra, J Broncano, R Ayala, M Ramos, V Llorente-Cortes, S Blasco-Turrión, F J Morales, P Gonzalez, R Brugada, A Mangas, R Toro
Idiopathic dilated cardiomyopathy has become one of the most prevalent inherited cardiomyopathies over the past decades. Genetic screening of first-degree relatives has revealed that 30-50% of the cases have a familial origin. Similar to other heart diseases, familial dilated cardiomyopathy is characterized by a high genetic heterogeneity that complicates family studies. Cli'nical screening, 12-lead electrocardiogram and transthoracic echocardiogram are recommended for patients and first-degree family members...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27886618/massive-parallel-sequencing-questions-the-pathogenic-role-of-missense-variants-in-dilated-cardiomyopathy
#20
Martin G Dalin, Pär G Engström, Emil G Ivarsson, Per Unneberg, Sara Light, Maria Schaufelberger, Thomas Gilljam, Bert Andersson, Martin O Bergo
BACKGROUND: Germline genetic variants are an important cause of dilated cardiomyopathy (DCM). However, recent sequencing studies have revealed rare variants in DCM-associated genes also in individuals without known heart disease. In this study, we investigate variant prevalence and genotype-phenotype correlations in Swedish DCM patients, and compare their genetic variants to those detected in reference cohorts. METHODS AND RESULTS: We sequenced the coding regions of 41 DCM-associated genes in 176 unrelated patients with idiopathic DCM and found 102 protein-altering variants with an allele frequency of <0...
February 1, 2017: International Journal of Cardiology
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