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idiopathic 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/28484840/epidemiology-of-ventricular-tachyarrhythmia-any-changes-in-the-past-decades
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28407464/practical-management-of-peripartum-cardiomyopathy
#14
REVIEW
Mi-Jeong Kim, Mi-Seung Shin
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy that causes systolic heart failure (HF) in previously healthy young women. Despite latest remarkable achievement, unifying pathophysiologic mechanism is not well established. Considering close temporal relationship to pregnancy, the recent prolactin theory is promising. Abnormal short form of 16-kDa prolactin may be produced in the oxidative stress milieu, show anti-angiogenic effect and damage cardiovascular structure in late pregnancy. Future study is needed to determine whether abnormal prolactin system is useful as a biomarker for diagnosis and therapy of PPCM...
May 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28301887/geburtshilfe-die-peripartale-kardiomyopathie
#15
Manuel Lingner, Jürgen Majolk, Oliver Niederer
The peripartum cardiomyopathy (PPCM) is an idiopathic heart failure secondary to left-ventricular dysfunction occuring at the end of pregnancy or in the months after delivery. Other causes for heart failure must not exist. The current state of knowledge about PPCM, based on a mini-literature-review, will be presented. We will focus on peripartal anaesthesiologic management and the case of a patient with respiratory insufficiency and lung edema secondary to PPCM.
March 2017: Anästhesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie: AINS
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
#16
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
#17
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
#18
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
#19
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
#20
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
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