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idiopathic dilated cardiomyopathy

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https://www.readbyqxmd.com/read/28902616/mef2c-loss-of-function-mutation-associated-with-familial-dilated-cardiomyopathy
#1
Fang Yuan, Zhao-Hui Qiu, Xing-Hua Wang, Yu-Min Sun, Jun Wang, Ruo-Gu Li, Hua Liu, Min Zhang, Hong-Yu Shi, Liang Zhao, Wei-Feng Jiang, Xu Liu, Xing-Biao Qiu, Xin-Kai Qu, Yi-Qing Yang
BACKGROUND: The MADS-box transcription factor myocyte enhancer factor 2C (MEF2C) is required for the cardiac development and postnatal adaptation and in mice-targeted disruption of the MEF2C gene results in dilated cardiomyopathy (DCM). However, in humans, the association of MEF2C variation with DCM remains to be investigated. METHODS: The coding regions and splicing boundaries of the MEF2C gene were sequenced in 172 unrelated patients with idiopathic DCM. The available close relatives of the index patient harboring an identified MEF2C mutation and 300 unrelated, ethnically matched healthy individuals used as controls were genotyped for MEF2C...
September 13, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28883920/exogenous-t3-toxicosis-following-consumption-of-a-contaminated-weight-loss-supplement
#2
R D'Arcy, M McDonnell, K Spence, C H Courtney
A 42-year-old male presented with a one-week history of palpitations and sweating episodes. The only significant history was of longstanding idiopathic dilated cardiomyopathy. Initial ECG demonstrated a sinus tachycardia. Thyroid function testing, undertaken as part of the diagnostic workup, revealed an un-measureable thyroid-stimulating hormone (TSH) and free thyroxine (T4). Upon questioning the patient reported classical thyrotoxic symptoms over the preceding weeks. Given the persistence of symptoms free tri-iodothyronine (T3) was measured and found to be markedly elevated at 48...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28868530/addressing-health-disparities-in-idiopathic-dilated-cardiomyopathy
#3
Argun D Can, Steven S Coughlin
No abstract text is available yet for this article.
June 2017: Jacobs Journal of Community Medicine
https://www.readbyqxmd.com/read/28859293/impaired-calcium-homeostasis-is-associated-with-sudden-cardiac-death-and-arrhythmias-in-a-genetic-equivalent-mouse-model-of-the-human-hrc-ser96ala-variant
#4
Christos Tzimas, Daniel M Johnson, Demetrio J Santiago, Elizabeth Vafiadaki, Demetrios A Arvanitis, Constantinos H Davos, Aimilia Varela, Nikolaos C Athanasiadis, Constantinos Dimitriou, Michalis Katsimpoulas, Stephan Sonntag, Mariya Kryzhanovska, Doron Shmerling, Stephan E Lehnart, Karin R Sipido, Evangelia G Kranias, Despina Sanoudou
Aims: The histidine-rich calcium-binding protein (HRC) Ser96Ala variant has previously been identified as a potential biomarker for ventricular arrhythmias and sudden cardiac death in patients with idiopathic dilated cardiomyopathy. Herein, the role of this variant in cardiac pathophysiology is delineated through a novel mouse model, carrying the human mutation in the homologous mouse position. Methods and results: The mouse HRC serine 81, homologous to human HRC serine 96, was mutated to alanine, using knock-in gene targeting...
September 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28808788/shocks-after-implantable-cardioverter-defibrillator-implantation-in-idiopathic-cardiomyopathy-patients-a-myocardial-biopsy-study
#5
Erdal Safak, Giuseppe D Ancona, Heinz-Peter Schultheiss, Uwe Kühl, Stephan Kische, Hilmi Kaplan, Hüseyin Ince, Jasmin Ortak
Prediction of follow-up shock is crucial to stratify patients with dilated cardiomyopathy (DCM) requiring implantable cardioverter defibrillator (ICD). The objective of the article is to assess the predictive value of endo-myocardial biopsy (EMB) towards ICD shock and follow-up mortality. A series of patients with DCM scheduled for ICD implantation underwent EMB to further determine the genesis of DCM. Presence of fibrosis and inflammation was documented and related to outcomes. A total of 240 patients were referred for ICD as primary (56%) and secondary (44%) prophylaxis...
August 14, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28803350/comparison-of-mitral-competence-after-mitral-repair-with-papillary-muscle-approximation-versus-papillary-muscle-relocation-for-functional-mitral-regurgitation
#6
Koji Furukawa, Mitsuhiro Yano, Eisaku Nakamura, Masakazu Matsuyama, Masanori Nishimura, Katsuya Kawagoe, Kunihide Nakamura
The purpose of this study was to evaluate the surgical results of papillary muscle approximation (PMA) and papillary muscle relocation (PMR) for functional mitral regurgitation (FMR) and to compare the effects of both procedures on the change in mitral regurgitation (MR) and echocardiogram parameters associated with tethering. Eighteen patients with moderate-to-severe FMR (MR grade ≥2) who underwent PMA or PMR were retrospectively analyzed. Underlying diseases were ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and aortic valve disease for seven, six, and five patients, respectively...
August 12, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28797313/intravenous-immunoglobulins-in-children-with-new-onset-dilated-cardiomyopathy
#7
Josephine F Heidendael, Suzanne L Den Boer, Joanne G Wildenbeest, Michiel Dalinghaus, Bart Straver, Dasja Pajkrt
BACKGROUND: Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded...
August 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28735322/viral-infection-of-the-heart-pathogenesis-and-diagnosis
#8
Mateusz Kuffner, Agnieszka Pawlak, Maciej Przybylski
Viral infections of the heart cause serious clinical problems, either as infectious myocarditis, which usually is a consequence of acute infection or as idiopathic dilated cardiomyopathy, resulting rather from a chronic infection. This minireview presents an up-to-date view on pathomechanisms of viral infection of the heart tissues, the role of immune system in controlling infectious process at its various stages and current possibilities of recognizing viral infection of the heart with use of both cardiological and virological methods...
January 2, 2017: Polish Journal of Microbiology
https://www.readbyqxmd.com/read/28701682/prognostic-impact-of-segmental-wall-motion-abnormality-in-patients-with-idiopathic-dilated-cardiomyopathy
#9
Yuichiro Iida, Takayuki Inomata, Toyoji Kaida, Teppei Fujita, Yuki Ikeda, Takeru Nabeta, Shunsuke Ishii, Toshimi Koitabashi, Ichiro Takeuchi, Junya Ako
The clinical impact of left ventricular (LV) segmental wall motion abnormalities (SWMA) in patients with idiopathic dilated cardiomyopathy (IDCM) has not been well elucidated.Among 100 consecutive IDCM patients with follow-up visits, we enrolled 85 after excluding those with left bundle branch block and/or ventricular pacemaker implantation. LV wall motion was assessed using left ventriculography scored for 7 segments according to the American Heart Association classification as follows: 0, normokinesis; 1, hypokinesis; 2, akinesis; and 3, dyskinesis...
August 3, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28696268/personalizing-risk-stratification-for-sudden-death-in-dilated-cardiomyopathy-the-past-present-and-future
#10
REVIEW
Brian P Halliday, John G F Cleland, Jeffrey J Goldberger, Sanjay K Prasad
Results from the DANISH Study (Danish Study to Assess the Efficacy of ICDs in Patients With Non-Ischemic Systolic Heat Failure on Mortality) suggest that for many patients with dilated cardiomyopathy (DCM), implantable cardioverter-defibrillators do not increase longevity. Accurate identification of patients who are more likely to die of an arrhythmia and less likely to die of other causes is required to ensure improvement in outcomes and wise use of resources. Until now, left ventricular ejection fraction has been used as a key criterion for selecting patients with DCM for an implantable cardioverter-defibrillator for primary prevention purposes...
July 11, 2017: Circulation
https://www.readbyqxmd.com/read/28663761/clinical-outcome-of-cardiac-resynchronization-therapy-in-dilated-phase-hypertrophic-cardiomyopathy
#11
Min Gu, Han Jin, Wei Hua, Xiao-Han Fan, Hong-Xia Niu, Tao Tian, Li-Gang Ding, Jing Wang, Cong Xue, Shu Zhang
BACKGROUNDS: Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-ischemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyopathy (DHCM) have been generally excluded from such trials. We aimed to compare the clinical outcome of CRT in patients with DHCM, idiopathic dilated cardiomyopathy (IDCM), or ischemic cardiomyopathy (ICM). METHODS: A total of 312 consecutive patients (DHCM: n = 16; IDCM: n = 231; ICM: n = 65) undergoing CRT in Fuwai hospital were studied respectively...
April 2017: Journal of Geriatric Cardiology: JGC
https://www.readbyqxmd.com/read/28614834/left-ventricular-mechanical-reverse-remodelling-not-followed-by-electrical-reverse-remodelling-a-case-report
#12
Sandra Amorim, Raquel Garcia, Teresa Pinho, João Rodrigues, Filipe Macedo, José Silva-Cardoso, M Júlia Maciel
Patients with severely depressed left ventricular ejection fractions (LVEFs) receive implantable cardioverter-defibrillators (ICDs) for the primary prevention of sudden death. However, in some patients, LVEFs may improve or even normalize over time, and these patients would no longer be qualified for ICD implantation based on the original criteria for which they have initially received an ICD. We report a patient with idiopathic dilated cardiomyopathy whose LVEF recovered to normal values after pharmacological therapy...
2017: Cardiology
https://www.readbyqxmd.com/read/28603826/differences-between-idiopathic-and-ischemic-dilated-cardiomyopathy
#13
Sana Fennira, Ihsen Zairi, Zouhaier Jnifene, Mariem Lakhal, Sofiene Kammoun, Sondos Kraiem
BACKGROUND: Ischemic cardiomyopathy can be reversible after revascularization hence the interest of making systematic coronary angiography that remain an invasive procedure. AIM: To detect epidemiological, clinical and paraclinical differences between idiopathic and ischemic dilated cardiomyopathy to identify predictors of coronary artery disease and to evaluate the interest of making systematic coronary angiography within the etiological check-up of dilated cardiomyopathy...
August 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28603002/association-of-preprocedural-cardiac-magnetic-resonance-imaging-with-outcomes-of-ventricular-tachycardia-ablation-in-patients-with-idiopathic-dilated-cardiomyopathy
#14
Konstantinos C Siontis, Hyungjin Myra Kim, Ghaith Sharaf Dabbagh, Rakesh Latchamsetty, Jadranka Stojanovska, Krit Jongnarangsin, Fred Morady, Frank M Bogun
BACKGROUND: Knowledge of complex arrhythmogenic substrates can help plan ventricular tachycardia (VT) ablation in patients with idiopathic dilated cardiomyopathy (DCM). OBJECTIVE: The purpose of this study was to assess whether preprocedural late gadolinium enhancement magnetic resonance imaging (LGE-MRI) can improve ablation outcomes in DCM. METHODS: Consecutive patients (N = 96) with idiopathic DCM underwent VT ablation with open-irrigated catheters (2006-2016)...
June 9, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28591770/inferolateral-early-repolarization-among-non-ischaemic-sudden-cardiac-death-victims
#15
Lauri T A Holmström, Mira A Haukilahti, Jani T Tikkanen, Aapo L Aro, Tuomas V Kenttä, Marja-Leena Kortelainen, Heikki V Huikuri, Matti J Junttila
Aim: Inferolateral early repolarization (ER) has been associated with an increased risk of sudden cardiac death (SCD). However, this association is thought to be mainly due to ischaemic SCD. The association of ER and non-ischaemic SCD has not been studied. The aim was to evaluate whether inferolateral ER is associated with non-ischaemic SCD. Methods and results: Study population consists of 275 consecutive victims of non-ischaemic SCD with 12-lead ECG and control group of general population cohort with 10 864 subjects...
June 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28567076/hereditary-dilated-cardiomyopathy-recent-advances-in-genetic-diagnostics
#16
REVIEW
Hyun-Young Park
Dilated cardiomyopathy (DCM) is the most common cause of heart failure in young adults and up to 50% of idiopathic DCM is thought to be caused by genetic mutations in candidate genes. Although a genetic diagnosis can confirm a clinical diagnosis of hereditary DCM, genetic testing has not been easily accessible due to genetic heterogeneity and complexity. Next-generation sequencing (NGS) technologies have recently been introduced, and genetic testing for multiple genes is currently available and more than 40 different genes have been associated with DCM...
May 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28557183/extracellular-vesicles-do-not-contribute-to-higher-circulating-levels-of-soluble-lrp1-in-idiopathic-dilated-cardiomyopathy
#17
Santiago Roura, Carolina Gálvez-Montón, David de Gonzalo-Calvo, Ana Gámez Valero, Paloma Gastelurrutia, Elena Revuelta-López, Cristina Prat-Vidal, Carolina Soler-Botija, Aida Llucià-Valldeperas, Isaac Perea-Gil, Oriol Iborra-Egea, Francesc E Borràs, Josep Lupón, Vicenta Llorente-Cortés, Antoni Bayes-Genis
Idiopathic dilated cardiomyopathy (IDCM) is a frequent cause of heart transplantation. Potentially valuable blood markers are being sought, and low-density lipoprotein receptor-related protein 1 (LRP1) has been linked to the underlying molecular basis of the disease. This study compared circulating levels of soluble LRP1 (sLRP1) in IDCM patients and healthy controls and elucidated whether sLRP1 is exported out of the myocardium through extracellular vesicles (EVs) to gain a better understanding of the pathogenesis of the disease...
May 29, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28539455/functional-consequences-of-rna-5-terminal-deletions-on-coxsackievirus-b3-rna-replication-and-ribonucleoprotein-complex-formation
#18
Nicolas Lévêque, Magali Garcia, Alexis Bouin, Joseph H C Nguyen, Genevieve P Tran, Laurent Andreoletti, Bert L Semler
Group B coxsackieviruses are responsible for chronic cardiac infections. However, the molecular mechanisms by which the virus can persist in the human heart long after the signs of acute myocarditis have abated are still not completely understood. Recently, coxsackievirus B3 strains with 5'-terminal deletions in genomic RNAs were isolated from a patient suffering from idiopathic dilated cardiomyopathy, suggesting that such mutant viruses may be the forms responsible for persistent infection. These deletions lacked portions of 5' stem-loop I, which is an RNA secondary structure required for viral RNA replication...
August 15, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28497865/predictors-and-implications-of-early-left-ventricular-ejection-fraction-improvement-in-new-onset-idiopathic-nonischemic-cardiomyopathy-with-narrow-qrs-complex-a-neolith-substudy
#19
Norman C Wang, Evan C Adelstein, Sandeep K Jain, Stuart G Mendenhall, Alaa A Shalaby, Andrew H Voigt, Samir Saba
BACKGROUND: Predictors and implications of early left ventricular ejection fraction (LVEF) improvement with guideline-directed medical therapy (GDMT) in new-onset idiopathic nonischemic cardiomyopathy (NICM) with narrow QRS complex are not well described. The objectives were to describe predictors of LVEF improvement after 3 months on GDMT and adverse cardiac events based on post-GDMT LVEF status (≤35% vs. >35%). METHODS: A retrospective cohort study was performed in subjects with new-onset NICM, LVEF ≤35%, and narrow QRS complex...
May 12, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28472305/recessive-taf1a-mutations-reveal-ribosomopathy-in-siblings-with-end-stage-pediatric-dilated-cardiomyopathy
#20
Pamela A Long, Jeanne L Theis, Yu-Huan Shih, Joseph J Maleszewski, Patrice C Abell Aleff, Jared M Evans, Xiaolei Xu, Timothy M Olson
Non-ischemic dilated cardiomyopathy (DCM) has been recognized as a heritable disorder for over 25 years, yet clinical genetic testing is non-diagnostic in >50% of patients, underscoring the ongoing need for DCM gene discovery. Here, whole exome sequencing uncovered a novel molecular basis for idiopathic end-stage heart failure in two sisters who underwent cardiac transplantation at three years of age. Compound heterozygous recessive mutations in TAF1A, encoding an RNA polymerase I complex protein, were associated with marked fibrosis of explanted hearts and gene-specific nucleolar segregation defects in cardiomyocytes, indicative of impaired ribosomal RNA synthesis...
August 1, 2017: Human Molecular Genetics
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