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idiopathic dilated cardiomyopathy

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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
#1
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
#2
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...
May 2, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28471539/sokolow-lyon-voltage-is-suitable-for-monitoring-improvement-in-cardiac-function-and-prognosis-of-patients-with-idiopathic-dilated-cardiomyopathy
#3
Kenji Fukaya, Kyosuke Takeshita, Takahiro Okumura, Hiroaki Hiraiwa, Soichiro Aoki, Takeo Ichii, Yuki Sugiura, Katsuhide Kitagawa, Toru Kondo, Naoki Watanabe, Naoaki Kano, Kenji Furusawa, Akinori Sawamura, Ryota Morimoto, Yasuko Bando, Toyoaki Murohara
BACKGROUND: The clinical significance of electrocardiogram in the assessment of patients with idiopathic dilated cardiomyopathy (IDCM) is currently unknown. The aim of this study was to determine the feasibility of recording serial changes in Sokolow-Lyon voltage (∆%QRS-voltage) in one year to estimate left ventricular reverse remodeling (LVRR) and predict a prognosis of IDCM patients under tailored medical therapy. METHODS: Sixty-eight consecutive patients with mild symptoms (52...
February 3, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28449952/heart-transplantation-in-giant-cell-myocarditis-analysis-of-the-united-network-for-organ-sharing-registry
#4
Chantal A Elamm, Sadeer G Al-Kindi, Christopher M Bianco, Bishnu P Dhakal, Guilherme H Oliveira
BACKGROUND: Giant cell myocarditis (GCM) is a lethal, rapidly progressive disease, for which heart transplantation (HTx) is the treatment of choice. We sought to describe the characteristics and outcomes of patients with GCM who undergo HTx. METHODS AND RESULTS: Methods and Results: We used the UNOS thoracic organ transplantation to identify adults with GCM as the primary diagnosis and compared their characteristics and outcomes with patients who were transplanted for other types of myocarditides and for idiopathic dilated cardiomyopathy (IDCMP)...
April 24, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28444133/increased-collagen-within-the-transverse-tubules-in-human-heart-failure
#5
David J Crossman, Xin Shen, Mia Jüllig, Michelle Munro, Yufeng Hou, Martin Middleditch, Darshan Shrestha, Amy Li, Sean Lal, Cristobal G Dos Remedios, David Baddeley, Peter N Ruygrok, Christian Soeller
Aims: In heart failure transverse-tubule (t-tubule) remodelling disrupts calcium release, and contraction. T-tubules in human failing hearts exhibit increased labelling by wheat germ agglutinin (WGA), a lectin that binds to the dystrophin-associated glycoprotein complex. We hypothesized changes in this complex may explain the increased WGA labelling and contribute to t-tubule remodelling in the failing human heart. In this study we sought to identify the molecules responsible for this increased WGA labelling...
April 20, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28438355/early-and-frequent-defibrillator-discharge-in-patients-with-cardiac-sarcoidosis-compared-with-patients-with-idiopathic-dilated-cardiomyopathy
#6
Yoichi Takaya, Kengo Kusano, Nobuhiro Nishii, Kazufumi Nakamura, Hiroshi Ito
BACKGROUND: Little is known about the suitability of implantable cardioverter defibrillator (ICD) implantation in patients with cardiac sarcoidosis (CS). We evaluated the efficacy of ICD implantation in patients with CS, including suspected CS, compared with those with idiopathic dilated cardiomyopathy (DCM). METHODS: A total of 102 consecutive patients with definite CS, suspected CS, or DCM who underwent ICD implantation were enrolled. The endpoint was the first documentation of appropriate ICD therapy for ventricular tachyarrhythmias...
April 19, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28290821/-detection-of-autoantibodies-against-the-1-adrenergic-receptor-in-the-sera-of-patients-via-the-competitive-cell-based-enzyme-linked-immunosorbent-assay
#7
A Y Shevelev, M V Kostiukevich, E E Efremov, T N Vlasik, N A Mironova, K A Zykov, N M Kashirina, I B Kuznetsova, T V Sharf, E N Mamochkina, L N Lipatova, M M Peklo, P N Rutkevich, E V Yanushevskaya, I N Rybalkin, O V Stukalova, T A Malkina, M M Belyaeva, T V Kuznetsova, G A Tkachev, L V Zinchenko, E M Gupalo, O Y Agapova, T V Yureneva-Tkhorzhevskaya, A V Rvacheva, M V Sidorova, A S Sadgyan, S N Tereshchenko, S P Golitsyn
OBJECTIVE: This study aimed to assess the level of anti-1-adrenergic receptor autoantibodies in patients with ventricular arrhythmias with no signs of organic heart disease and with presence of cardiovascular pathology in comparison with a group of healthy volunteers. MATERIAL AND METHODS: The study included 44 patients with ventricular arrhythmias with no signs of organic heart disease ("idiopathic"), 34 patients with diagnosed dilated cardiomyopathy (DCM) of inflammatory origin, 35 patients with coronary heart disease and ventricular arrhythmias, 12patients with coronary heart disease with no ventricular arrhythmias, and 19 healthy volunteers (control group)...
December 2016: Kardiologiia
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#8
REVIEW
Imre J Pavo, Ina Michel-Behnke
Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients...
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28288703/erratum-to-prevalence-predictors-and-prognosis-of-ventricular-reverse-remodeling-in-idiopathic-dilated-cardiomyopathy-rev-port-cardiol-2016-35-5-253-260
#9
Sandra Amorim, Manuel Campelo, Elisabete Martins, Brenda Moura, Alexandra Sousa, Teresa Pinho, José Silva-Cardoso, Maria Júlia Maciel
No abstract text is available yet for this article.
March 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28283502/validation-of-noninvasive-measurement-of-cardiac-output-using-inert-gas-rebreathing-in-a-cohort-of-patients-with-heart-failure-and-reduced-ejection-fraction
#10
Mohamed Hassan, Kerolos Wagdy, Ahmed Kharabish, Peter Philip Selwanos, Ahmed Nabil, Ahmed Elguindy, Amr ElFaramawy, Mahmoud F Elmahdy, Hani Mahmoud, Magdi H Yacoub
BACKGROUND: Cardiac output (CO) is a key indicator of cardiac function in patients with heart failure. No completely accurate method is available for measuring CO in all patients. The objective of this study was to validate CO measurement using the inert gas rebreathing (IGR) method against other noninvasive and invasive methods of CO quantification in a cohort of patients with heart failure and reduced ejection fraction. METHODS AND RESULTS: The study included 97 patients with heart failure and reduced ejection fraction (age 42±15...
March 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28258649/the-cardiac-manifestations-of-inherited-metabolic-diseases-in-children
#11
REVIEW
David F A Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEMs) are responsible for around 5% of all cases of cardiomyopathy (CM) and 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic cardiomyopathy, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations. IEMs such as Barth syndrome, a disorder of cardiolipin stability usually associated with DCM, have been associated with rarer types of CM such as endocardial fibroelastosis (EFE) and left ventricular non-compaction...
March 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28229198/-catheter-ablation-of-ventricular-tachycardia-what-have-we-achieved
#12
T Konrad, B A Hoffmann, T Rostock
The role of catheter ablation in patients with ventricular tachycardia (VT) has evolved over the last two decades into an established treatment option. In patients with idiopathic VT catheter ablation is the gold standard treatment option with high effectiveness and low risk of complications. Due to the high risk of side effects the use of antiarrhythmic drugs is only indicated in exceptional cases. In patients with structural heart diseases, such as ischemic and dilated cardiomyopathy, VT is the most frequent cause of death...
February 22, 2017: Herz
https://www.readbyqxmd.com/read/28225712/left-ventricular-function-and-exercise-performance-in-idiopathic-dilated-cardiomyopathy-role-of-tissue-doppler-imaging
#13
Elena Zambon, Annamaria Iorio, Concetta Di Nora, Cosimo Carriere, Elena Abate, Marco Merlo, Giulia Barbati, Andrea Di Lenarda, Bruno Pinamonti, Piergiuseppe Agostoni, Gianfranco Sinagra
BACKGROUND: To examine the relationship between left ventricular (LV) function evaluated at echocardiography and exercise performance in idiopathic dilated cardiomyopathy (IDCM) patients. METHODS AND RESULTS: We enrolled 76 consecutive IDCM patients in sinus rhythm, undergoing cardiopulmonary exercise testing and echocardiography [49 ± 13 years old; LV ejection fraction 31 ± 7%, LV end-diastolic volume 96 ± 31 ml/m; peak oxygen consumption (peak VO2/kg) 18 ± 5...
April 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28224037/tooth-extraction-in-a-patient-after-autologous-skeletal-myoblast-sheet-transplantation-for-severe-dilated-cardiomyopathy
#14
Kohei Okuyama, Yuki Sakamoto, Tomofumi Naruse, Souichi Yanamoto, Masahiro Umeda
The authors describe the first case of tooth extraction in a patient treated with autologous skeletal myocardial sheet transplantation for severe dilated cardiomyopathy. This condition is associated with significant morbidity and mortality that may have identifiable causes or may be idiopathic or inherited. In addition to local disinfection, antimicrobial prophylaxis and continuous anticoagulant therapy during the perioperative period of tooth extraction were needed to prevent infective endocarditis and ensure the success of regenerative therapy of the heart...
March 2017: Annals of Medicine and Surgery
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
#15
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...
April 11, 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
#16
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
#17
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
#18
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
#19
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
#20
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
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