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idiopathic dilated cardiomyopathy

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https://www.readbyqxmd.com/read/29311597/putative-functional-genes-in-idiopathic-dilated-cardiomyopathy
#1
Nishanth Ulhas Nair, Avinash Das, Uri Amit, Welles Robinson, Seung Gu Park, Mahashweta Basu, Alex Lugo, Jonathan Leor, Eytan Ruppin, Sridhar Hannenhalli
Idiopathic dilated cardiomyopathy (DCM) is a complex disorder with a genetic and an environmental component involving multiple genes, many of which are yet to be discovered. We integrate genetic, epigenetic, transcriptomic, phenotypic, and evolutionary features into a method - Hridaya, to infer putative functional genes underlying DCM in a genome-wide fashion, using 213 human heart genomes and transcriptomes. Many genes identified by Hridaya are experimentally shown to cause cardiac complications. We validate the top predicted genes, via five different genome-wide analyses: First, the predicted genes are associated with cardiovascular functions...
January 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29305677/coupling-interval-variability-of-premature-ventricular-contractions-in-patients-with-different-underlying-pathology-an-insight-into-the-arrhythmia-mechanism
#2
Lennart J de Vries, Mihran Martirosyan, Ron T van Domburg, Sip A Wijchers, Tamas Géczy, Tamas Szili-Torok
PURPOSE: Coupling interval (CI) variability of premature ventricular contractions (PVCs) is influenced by the underlying arrhythmia mechanism. The aim of this study was to compare CI variability of PVCs in different myocardial disease entities, in order to gain insight into their arrhythmia mechanism. METHODS: Sixty-four patients with four underlying pathologies were included: idiopathic (n = 16), non-ischemic dilated cardiomyopathy (NIDCM) (n = 16), familial cardiomyopathy (PLN/LMNA) (n = 16), and post-MI (n = 16)-associated PVCs...
January 5, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29285418/pdk4-deficiency-induces-intrinsic-apoptosis-in-response-to-starvation-in-fibroblasts-from-doberman-pinschers-with-dilated-cardiomyopathy
#3
Kathryn Taggart, Amara Estrada, Patrick Thompson, Francisco Lourenco, Sara Kirmani, Silveli Suzuki-Hatano, Christina A Pacak
The Doberman pinscher (DP) canine breed displays a high incidence of idiopathic, nonischemic dilated cardiomyopathy (DCM) with increased mortality. A common mutation in DPs is a splice site deletion in the pyruvate dehydrogenase kinase 4 (PDK4) gene that shows a positive correlation with DCM development. PDK4, a vital mitochondrial protein, controls the switch between glycolysis and oxidative phosphorylation based upon nutrient availability. It is likely, although unproven, that DPs with the PDK4 mutation are unable to switch to oxidative phosphorylation during periods of low nutrient availability, and thus are highly susceptible to mitochondrial-mediated apoptosis...
2017: BioResearch Open Access
https://www.readbyqxmd.com/read/29274800/cardiac-resynchronization-therapy-device-implantation-in-a-patient-with-persistent-left-superior-vena-cava-without-communicating-innominate-vein-should-we-proceed-from-the-same-side-a-dilemma-revisited
#4
Debabrata Bera, Neeta Bachani, Prashant Pawar, Chetan Rathi, Vadivelu Ramalingam, Yash Lokhandwala
Persistent left superior vena cava (PLSVC) is an uncommon congenital anomaly. We report a case of implantation of cardiac resynchronization therapy - pacemaker (CRT-P) device in a 38-year-old lady with idiopathic dilated cardiomyopathy. After left axillary vein puncture, we faced an unexpected entry of left subclavian to PLSVC draining into the coronary sinus (CS). The target posterolateral vein which had been identified before seemed to have an acute angle at its entry into the CS. Hence, at this stage we were in a dilemma, whether to switch to the right side or to continue from the same side...
December 21, 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29258998/contractile-reserve-as-a-predictor-of-prognosis-in-patients-with-non-ischaemic-systolic-heart-failure-and-dilated-cardiomyopathy-a-systematic-review-and-meta-analysis
#5
Peter H Waddingham, Sanjeev Bhattacharyya, Jet Van Zalen, Guy Lloyd
OBJECTIVE: Patients with non-ischaemic systolic heart failure (HF) and idiopathic dilated cardiomyopathy (DCM) are a heterogenous group with varied morbidity and mortality. Prognostication in this group is challenging. We performed a systematic review and meta-analysis to examine the significance of the presence of contractile reserve as assessed via stress imaging on mortality and hospitalisation. METHODS: A search for studies that non-invasively assessed contractile reserve in patients with DCM or non-ischaemic HF with reduced ejection fraction, stress imaging with follow-up data comparing outcomes...
March 2018: Echo Research and Practice
https://www.readbyqxmd.com/read/29237686/toward-genetics-driven-early-intervention-in-dilated-cardiomyopathy-design-and-implementation-of-the-dcm-precision-medicine-study
#6
REVIEW
Daniel D Kinnamon, Ana Morales, Deborah J Bowen, Wylie Burke, Ray E Hershberger
BACKGROUND: The cause of idiopathic dilated cardiomyopathy (DCM) is unknown by definition, but its familial subtype is considered to have a genetic component. We hypothesize that most idiopathic DCM, whether familial or nonfamilial, has a genetic basis, in which case a genetics-driven approach to identifying at-risk family members for clinical screening and early intervention could reduce morbidity and mortality. METHODS: On the basis of this hypothesis, we have launched the National Heart, Lung, and Blood Institute- and National Human Genome Research Institute-funded DCM Precision Medicine Study, which aims to enroll 1300 individuals (600 non-Hispanic African ancestry, 600 non-Hispanic European ancestry, and 100 Hispanic) who meet rigorous clinical criteria for idiopathic DCM along with 2600 of their relatives...
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29233129/basal-wall-hypercontraction-of-takotsubo-cardiomyopathy-in-a-patient-who-had-been-diagnosed-with-dilated-cardiomyopathy-a-case-report
#7
Noboru Ichihara, Shuichi Fujita, Yumiko Kanzaki, Tomohiro Fujisaka, Michishige Ozeki, Nobukazu Ishizaka
BACKGROUND: Takotsubo cardiomyopathy is characterized by the basal hypercontractility and apical ballooning of the left ventriculum and T-wave inversion in the electrocardiogram. It has been suggested that Takotsubo cardiomyopathy might underlie the pathogenesis of persistent cardiac dysfunction; however, few reports are present demonstrating the advent of Takotsubo cardiomyopathy in patients with idiopathic cardiomyopathy. CASE PRESENTATION: A 64-year-old women was admitted due to dyspnea on effort and lower extremity edema...
December 12, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29224255/the-highest-mortality-rates-in-childhood-dilated-cardiomyopathy-occur-during-the-first-year-after-diagnosis
#8
Shalan Fadl, Håkan Wåhlander, Katja Fall, Yang Cao, Jan Sunnegårdh
AIM: The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and non-compaction of the left ventricle (LVNC) in Swedish children. METHODS: We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular non-compaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015. RESULTS: In total, 69 cases (61% males) were identified...
December 9, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29208596/urinary-8-hydroxy-2-deoxyguanosine-as-a-myocardial-oxidative-stress-marker-is-associated-with-ventricular-tachycardia-in-patients-with-active-cardiac-sarcoidosis
#9
Hironori Ishiguchi, Shigeki Kobayashi, Takeki Myoren, Michiaki Kohno, Takuma Nanno, Wakako Murakami, Seiko Oda, Keishi Oishi, Shinichi Okuda, Munemasa Okada, Kazuyoshi Suga, Masafumi Yano
BACKGROUND: Recently, we reported that urinary 8-hydroxy-2'-deoxyguanosine (U-8-OHdG), an oxidative stress marker, reflected inflammatory activity in cardiac sarcoidosis (CS). Here, we investigated whether U-8-OHdG levels were associated with ventricular tachycardia (VT) in patients with CS. METHODS AND RESULTS: This prospective cohort study enrolled 62 consecutive patients with CS, of whom 36 were diagnosed as having active CS based on abnormal 18F-flurodeoxyglucose accumulation in the heart on positron-emission tomography/computed tomography...
December 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29203463/contractile-reserve-as-a-predictor-of-prognosis-in-non-ischaemic-systolic-heart-failure-a-systematic-review-and-meta-analysis
#10
Peter H Waddingham, Sanjeev Bhattacharyya, Jet Van Zalen, Guy Lloyd
OBJECTIVE: Patients with non-ischaemic systolic heart failure (HF) and idiopathic dilated cardiomyopathy (DCM) are a heterogenous group with varied morbidity and mortality. Prognostication in this group is challenging. We performed a systematic review and meta-analysis to examine the significance of the presence of contractile reserve as assessed via stress imaging on mortality and hospitalisation. METHODS: A search for studies that non-invasively assessed contractile reserve in patients with DCM or non-ischaemic HF with reduced ejection fraction; stress imaging with follow up data comparing outcomes...
December 4, 2017: Echo Research and Practice
https://www.readbyqxmd.com/read/29169474/survival-without-cardiac-transplantation-among-children-with-dilated%C3%A2-cardiomyopathy
#11
Rakesh K Singh, Charles E Canter, Ling Shi, Steven D Colan, Debra A Dodd, Melanie D Everitt, Daphne T Hsu, John L Jefferies, Paul F Kantor, Elfriede Pahl, Joseph W Rossano, Jeffrey A Towbin, James D Wilkinson, Steven E Lipshultz
BACKGROUND: Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. OBJECTIVES: This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. METHODS: Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009)...
November 28, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29154888/activation-of-yap1-taz-signaling-in-ischemic-heart-disease-and-dilated-cardiomyopathy
#12
Ning Hou, Ying Wen, Xun Yuan, Haodong Xu, Xuejun Wang, Faqian Li, Bo Ye
Genetic manipulation of key components of the evolutionally conserved Hippo pathway has shown that the precise control of these signaling molecules is critical to cardiac development and response to stresses. However, how this pathway is involved in the progression of cardiac dysfunction in different heart diseases remains unclear. We investigated the expressional levels and subcellular localization of Yap1, Taz, and Tead1 and determined Hippo target gene expression in failing human hearts with ischemic heart disease (IHD) and idiopathic dilated cardiomyopathy (IDC) and mouse desmin-related cardiomyopathy (DES)...
December 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29148208/comparison-of-long-term-outcome-in-anthracycline-related-versus-idiopathic-dilated-cardiomyopathy-a-single-centre-experience
#13
Alessandra Fornaro, Iacopo Olivotto, Luigi Rigacci, Mauro Ciaccheri, Benedetta Tomberli, Cecilia Ferrantini, Raffaele Coppini, Francesca Girolami, Francesco Mazzarotto, Marco Chiostri, Massimo Milli, Niccolò Marchionni, Gabriele Castelli
AIMS: Cardiac dysfunction is a severe complication of anthracycline-containing anticancer therapy. The outcome of anthracycline-induced cardiomyopathy (AICM) compared with other non-ischaemic causes of heart failure (HF), such as idiopathic dilated cardiomyopathy (IDCM), is unresolved. The aim of this study was to compare the survival of AICM patients with an IDCM cohort followed at our centre from 1990 to 2016. METHODS AND RESULTS: We included 67 patients (67% female, 50 ± 15 years) with AICM, defined as onset of otherwise unexplained left ventricular ejection fraction (LVEF) ≤50% following anthracycline therapy, and 488 IDCM patients (28% female, 55 ± 12 years)...
November 16, 2017: European Journal of Heart Failure
https://www.readbyqxmd.com/read/29141071/usefulness-of-total-12-lead-qrs-voltage-as-a-clue-to-diagnosis-of-patients-with-cardiac-sarcoidosis-severe-enough-to-warrant-orthotopic-heart-transplant
#14
William C Roberts, Tiffany M Becker, Shelley A Hall
Importance: Severe heart failure caused by cardiac sarcoidosis is difficult to diagnosis without biopsy. Objective: To assess whether total electrocardiographic 12-lead QRS voltage may be a clue to diagnosis. Design, Setting, Participants: Case-series study with cases collected at Baylor University Medical Center at Dallas, Dallas, Texas, from January 13, 2005, to January 24, 2017. The clinical records of 16 patients with severe heart failure caused by cardiac sarcoidosis were studied...
November 15, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/29127129/heart-failure-in-dilated-cardiomyopathy-mimicking-asthma-triggered-by-pneumonia
#15
Kenichi Tetsuhara, Satoshi Tsuji, Katsutoshi Nakano, Mitsuru Kubota
Heart failure is a rare cause of wheezing and may develop into a critical condition in children. Few cases report patients with heart failure, secondary to dilated cardiomyopathy, with high fever. A 23-month-old girl visited the emergency department with high fever, cough, first wheezing episode, chest retraction and tachycardia. The chest X-ray revealed consolidation on the left lower lung field; the cardiothoracic ratio was 60%. She was diagnosed with bronchial asthma triggered by pneumonia, which remained unchanged during four visits...
November 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29121733/pheochromocytoma-as-a-reversible-cause-of-cardiomyopathy-analysis-and-review-of-the-literature
#16
Rong Zhang, Deepashree Gupta, Stewart G Albert
CONTEXT: Pheochromocytoma and paraganglioma are rare neuroendocrine tumors which overproduce catecholamines and arise from the adrenal gland or extra-adrenal chromaffin cells of the sympathetic and parasympathetic ganglia (1). Excessive catecholamine-induced stimulation of cardiac myocytes leads to damage which manifests in several forms ranging from Takotsubo to dilated cardiomyopathy. Diagnosis of pheochromocytoma-related cardiomyopathies is often delayed due to the atypical presentation associated with many cases...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29115553/endothelial%C3%A2-to%C3%A2-mesenchymal-transition-in-human-idiopathic-dilated-cardiomyopathy
#17
Yeqing Xie, Jianquan Liao, Yong Yu, Qi Guo, Yingzhen Yang, Junbo Ge, Haozhu Chen, Ruizhen Chen
Dilated cardiomyopathy (DCM) is characterized by left ventricular dilation and cardiac fibrosis. Emerging evidence indicated that endothelial‑to‑mesenchymal transition (Endo‑MT) is a crucial event during organ fibrosis. This study was performed to clarify whether Endo‑MT contributed to the progression of cardiac fibrosis in DCM. Cardiac samples from patients with DCM and control were obtained. The presence of endothelial markers, cluster of differentiation (CD)31 and vascular endothelial (VE)‑cadherin, and mesenchymal markers, α smooth muscle actin (SMA) and fibroblast‑specific protein 1 (FSP1) was performed using immunohistochemistry...
November 8, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29106559/outcome-of-heart-transplantation-after-bridge-to-transplant-strategy-using-various-mechanical-circulatory-support-devices
#18
Daisuke Yoshioka, Boyangzi Li, Hiroo Takayama, Reshad A Garan, Veli K Topkara, Jiho Han, Paul Kurlansky, Melana Yuzefpolskaya, Paolo C Colombo, Yoshifumi Naka, Koji Takeda
OBJECTIVES: Orthotopic heart transplantation (OHT) is limited by a chronic shortage of donors. With the evolution of technology, more patients have been bridged to transplant (BTT) through various pathways using various types of mechanical circulatory support. We compared short- and long-term outcomes among these various strategies of BTT. METHODS: We retrospectively reviewed 410 patients who had OHT between January 2009 and April 2015. Patients were divided into 4 groups according to BTT status: primary OHT without bridging (Group A, n  = 246); bridge with implantable continuous-flow left ventricular assist device (CF-LVAD) (Group B, n  = 130); bridge with short-term mechanical circulatory support (Group C, n  = 16) and bridge with multiple mechanical circulatory supports, including short-term mechanical circulatory support and CF-LVAD (Group D, n  = 18)...
July 4, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29039828/-clinical-laboratory-and-instrumental-criteria-for-myocarditis-established-in-comparison-with-myocardial-biopsy-a-non-invasive-diagnostic-algorithm
#19
O V Blagova, Yu V Osipova, A V Nedostup, E A Kogan, V A Sulimov
AIM: To determine the diagnostic value of different clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in patients with the picture of idiopathic arrhythmias, dilated cardiomyopathy (DCM) and in a comparison group when comparing with myocardial morphological examination. SUBJECTS AND METHODS: A study group included 100 patients (35 women; mean age, 44.7±12.5 years) with idiopathic arrhythmias (n=20) and DCM as a syndrome (n=100). All underwent myocardial morphological examination: endomyocardial biopsy (EMB) (n=71), intraoperative biopsy (n=13), study of the explanted heart (n=6), and autopsy (n=11)...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28978409/specific-indications-and-clinical-outcome-in-patients-with-subcutaneous-icd-a-nationwide-multicentre-registry
#20
Michael Sponder, Cesar Khazen, Wolfgang Dichtl, Lukas Fiedler, Deddo Mörtl, Alexander Teubl, Clemens Steinwender, Martin Martinek, Michael Nürnberg, Daniel Dalos, Johannes Kastner, Christoph Schukro
BACKGROUND: Subcutaneous implantable cardioverter-defibrillators (S-ICD) are an innovative and less invasive alternative to transvenous ICD (TV-ICD) in selected patients. We aimed to investigate the underlying diseases and the specific indications for implanting S-ICD in clinical practice, as well as the prevalence of shock delivery and complications. METHODS AND RESULTS: From December 2012, data of 236 patients (30,5% female; age 48,6±16,8years) were gathered from 12 centres in Austria...
October 1, 2017: European Journal of Internal Medicine
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