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idiopathic cardiomyopathy

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https://www.readbyqxmd.com/read/28614834/left-ventricular-mechanical-reverse-remodelling-not-followed-by-electrical-reverse-remodelling-a-case-report
#1
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...
June 15, 2017: Cardiology
https://www.readbyqxmd.com/read/28603826/differences-between-idiopathic-and-ischemic-dilated-cardiomyopathy
#2
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-pre-procedural-cardiac-magnetic-resonance-imaging-with-outcomes-of-ventricular-tachycardia-ablation-in-patients-with-idiopathic-dilated-cardiomyopathy
#3
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 idiopathic dilated cardiomyopathy (DCM). OBJECTIVE: We sought to assess whether pre-procedural 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). Before 2012, LGE-MRI was not performed at our institution in patients with implanted devices, but it has been performed routinely in all patients after the implementation of a new MRI protocol in 2012...
June 8, 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
#4
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
#5
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
#6
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
#7
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...
May 24, 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
#8
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/28484840/epidemiology-of-ventricular-tachyarrhythmia-any-changes-in-the-past-decades
#9
REVIEW
Benjamin Jong-Ming Pang, Martin Stephen Green
Ventricular tachyarrhythmias include potentially lethal episodes of sustained ventricular tachycardia (VT) and ventricular fibrillation (VF) as well as hemodynamically tolerated ventricular ectopic activity. Sustained VT or VF may develop in the setting of acute myocardial infarction or as clinical sequelae of advanced cardiomyopathy. The incidence of these serious arrhythmias is estimated from retrospective and observational studies and registries of sudden cardiac arrest and sudden cardiac death. Over the past few decades, there has been a gradual decline in the incidence of life-threatening ventricular tachyarrhythmias which has been largely driven by upstream treatments for and prevention of coronary artery disease and its sequelae...
May 8, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28472305/recessive-taf1a-mutations-reveal-ribosomopathy-in-siblings-with-end-stage-pediatric-dilated-cardiomyopathy
#10
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/28471885/extracorporeal-membrane-oxygenation-for-acute-decompensated-heart-failure
#11
Laurence Dangers, Nicholas Bréchot, Matthieu Schmidt, Guillaume Lebreton, Guillaume Hékimian, Ania Nieszkowska, Sébastien Besset, Jean-Louis Trouillet, Jean Chastre, Pascal Leprince, Alain Combes, Charles-Edouard Luyt
OBJECTIVE: Long-term outcomes of patients treated with venoarterial-extracorporeal membrane oxygenation for acute decompensated heart failure (i.e., cardiogenic shock complicating chronic cardiomyopathy) have not yet been reported. This study was undertaken to describe their outcomes and determine mortality-associated factors. DESIGN: Retrospective analysis of data prospectively collected. SETTING: Twenty-six-bed tertiary hospital ICU. PATIENTS: One hundred five patients implanted with venoarterial-extracorporeal membrane oxygenation for acute decompensated heart failure...
May 3, 2017: Critical Care Medicine
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
#12
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/28469493/beyond-the-electrocardiogram-mutations-in-cardiac-ion-channel-genes-underlie-nonarrhythmic-phenotypes
#13
REVIEW
Thomas M Roston, Taylor Cunningham, Anna Lehman, Zachary W Laksman, Andrew D Krahn, Shubhayan Sanatani
Cardiac ion channelopathies are an important cause of sudden death in the young and include long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, and short QT syndrome. Genes that encode ion channels have been implicated in all of these conditions, leading to the widespread implementation of genetic testing for suspected channelopathies. Over the past half-century, researchers have also identified systemic pathologies that extend beyond the arrhythmic phenotype in patients with ion channel gene mutations, including deafness, epilepsy, cardiomyopathy, periodic paralysis, and congenital heart disease...
2017: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/28458898/efficacy-of-pasireotide-in-controlling-severe-hypercortisolism-until-cardiac-transplantation
#14
Roberto Attanasio, Liana Cortesi, Daniela Gianola, Claudia Vettori, Fulvio Sileo, Roberto Trevisan
SUMMARY: Cushing's syndrome is associated with increased morbidity and mortality. Although surgery is the first-line treatment, drugs can still play a role as an ancillary treatment to be employed while waiting for surgery, after unsuccessful operation or in patients unsuitable for surgery. We were asked to evaluate a 32-year-old male waiting for cardiac transplantation. Idiopathic hypokinetic cardiomyopathy had been diagnosed since 6 years. He was on treatment with multiple drugs, had a pacemaker, an implantable cardioverter and an external device for the support of systolic function...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28453795/closing-the-gap-in-paediatric-ventricular-assist-device-therapy-with-the-berlin-heart-excor%C3%A2-15-ml-pump%C3%A2
#15
Fabrizio De Rita, Massimo Griselli, Eugen Sandica, Oliver Miera, Ann Karimova, Yves d'Udekem, Ranny Goldwasser, Katarzyna Januszewska, Antonio Amodeo, Nadine Jurrmann, Simon Ersel, Ares K Menon
OBJECTIVES: The Berlin Heart EXCOR ® (EXCOR) paediatric ventricular assist device is used worldwide for mechanical support of infants and small children with end-stage heart failure. A clinically important gap between the smallest EXCOR blood pump (10 ml) and the next larger size (25 ml) limited the choice of pump size in patients with a body surface area (BSA) between 0.33 and 0.5 m 2 . We present the first clinical experience from the early product surveillance (EPS) of the new EXCOR 15-ml blood pump...
May 1, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28451455/metabolic-profiles-in-heart-failure-due-to-non-ischemic-cardiomyopathy-at-rest-and-under-exercise
#16
Matthias Mueller-Hennessen, Johanna Sigl, Jens C Fuhrmann, Henning Witt, Regina Reszka, Oliver Schmitz, Jürgen Kastler, Jenny J Fischer, Oliver J Müller, Evangelos Giannitsis, Tanja Weis, Norbert Frey, Hugo A Katus
AIMS: Identification of metabolic signatures in heart failure (HF) patients and evaluation of their diagnostic potential to discriminate HF patients from healthy controls during baseline and exercise conditions. METHODS: Plasma samples were collected from 22 male HF patients with non-ischemic idiopathic cardiomyopathy and left ventricular systolic dysfunction and 19 healthy controls before (t0), at peak (t1) and 1 h after (t2) symptom-limited cardiopulmonary exercise testing...
May 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28449952/heart-transplantation-in-giant-cell-myocarditis-analysis-of-the-united-network-for-organ-sharing-registry
#17
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
#18
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
#19
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/28431055/electrocardiographic-differentiation-of-idiopathic-right-ventricular-outflow-tract-ectopy-from-early-arrhythmogenic-right-ventricular-cardiomyopathy
#20
Jan Novak, Alessandro Zorzi, Silvia Castelletti, Antonis Pantasis, Ilaria Rigato, Domenico Corrado, William Mckenna, Pier D Lambiase
Aims: The differentiation between idiopathic right ventricular outflow tract (RVOT) arrhythmias and early arrhythmogenic right ventricular cardiomyopathy (ARVC) can be challenging. We aimed to assess whether QRS morphological features and coupling interval of ventricular ectopic beats (VEBs) can improve differentiation between the two conditions. Methods and Results: Twenty desmosomal-gene mutation carriers (13 females, mean age 43 years) with no or mild ARVC phenotypic expression and 33 age- and sex-matched subjects with idiopathic RVOT arrhythmias were studied...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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