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Cardiomyopathies

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https://www.readbyqxmd.com/read/29668027/heart-transplant-in-children-what-a-primary-care-provider-needs-to-know
#1
Swati Sehgal, Emily Shea, Lauren Kelm, Deepak Kamat
Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant...
April 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29667903/posterior-mitral-leaflet-plication-for-hypertrophic-obstructive-cardiomyopathy
#2
Praveen Kerala Varma, Neethu Krishna, Hisham Ahamed, Sujatha Madassery
Anomalies of the mitral valve apparatus in hypertrophic cardiomyopathy are an important cause of systolic anterior motion. Patients with significant residual obstruction due to systolic anterior motion after myectomy and anterior mitral leaflet plication may end up having mitral valve replacement. We describe the case of a 52-year-old man who underwent posterior mitral leaflet plication to correct residual systolic anterior motion after anterior mitral leaflet plication.
January 1, 2018: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29667349/asb1-differential-methylation-in-ischaemic-cardiomyopathy-relationship-with-left-ventricular-performance-in-end-stage-heart-failure-patients
#3
Ana Ortega, Estefanía Tarazón, Carolina Gil-Cayuela, Luis Martínez-Dolz, Francisca Lago, José Ramón González-Juanatey, Juan Sandoval, Manuel Portolés, Esther Roselló-Lletí, Miguel Rivera
AIMS: Ischaemic cardiomyopathy (ICM) leads to impaired contraction and ventricular dysfunction, causing high rates of morbidity and mortality. Epigenomics allows the identification of epigenetic signatures in human diseases. We analyse the differential epigenetic patterns of the ASB gene family in ICM patients and relate these alterations to their haemodynamic and functional status. METHODS AND RESULTS: Epigenomic analysis was carried out using 16 left ventricular (LV) tissue samples, eight from ICM patients undergoing heart transplantation and eight from control (CNT) subjects without cardiac disease...
April 17, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29667277/arrhythmic-manifestation-of-peripartum-cardiomyopathy-case-report
#4
Patryk Siedlecki, Małgorzata Kurpesa, Barbara Uznańska-Loch, Ewa Trzos, Jarosław Damian Kasprzak
We report a case of 26-year-old woman, with arrhythmic manifestation of peripartum cardiomyopathy with moderate heart failure. Ventricular arrhythmia recorded in ambulatory Holter ECG (premature ventricular contractions) was most severe during pregnancy, reduced when beta-blocker therapy was initiated and almost completely resolved after delivery. Then, 1 year after hospitalization in Cardiology Department, recurrence was observed with recorded short episode of nonsustained ventricular tachycardia.
April 17, 2018: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29667276/stress-cardiomyopathy-provoked-chaotic-t-wave-lability
#5
Soheila Talebi, Alaleh Azhir, Edgar Argulian
Potentially lethal arrhythmia, occurring at presentation or during hospitalization, are a part of the clinical spectrum of stress cardiomyopathy. There is significant association between the catecholamine provoked nonalternating beat to beat fluctuations in T-wave morphology, termed T-wave lability (TWL), and the clinical risk of arrhythmia. We report four patients with diagnosis of stress cardiomyopathy, in whom serial electrocardiography showed macrovoltage nonalternating fluctuations in T-wave morphology...
April 17, 2018: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29666464/an-adolescence-onset-male-leukoencephalopathy-with-remarkable-cerebellar-atrophy-and-novel-compound-heterozygous-aars2-gene-mutations-a-case-report
#6
Qing Dong, Ling Long, Yan-Yu Chang, Yan-Jun Lin, Mei Liu, Zheng-Qi Lu
Mutations in the mitochondrial alanyl-transfer (t)RNA synthetase 2 (AARS2; OMIM 612035) have been linked to leukoencephalopathy recently. Until now, there have been only 13 cases reported in the literature. Hence, the clinical and genetic characteristics of this disease are not fully understood. Here, we reported an adolescence-onset male leukoencephalopathy patient characterized by progressive limb tremor at the age of 17 years. He had no signs of a cardiomyopathy. Magnetic resonance imaging scanning demonstrated severe cerebellar atrophy and white matter abnormalities involving descending tracts...
April 17, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29666415/canonical-pi3k%C3%AE-signaling-in-myeloid-cells-restricts-trypanosoma-cruzi-infection-and-dampens-chagasic-myocarditis
#7
Maria C Silva, Marcela Davoli-Ferreira, Tiago S Medina, Renata Sesti-Costa, Grace K Silva, Carla D Lopes, Lucas E Cardozo, Fábio N Gava, Konstantina Lyroni, Fabrício C Dias, Amanda F Frade, Monique Baron, Helder I Nakaya, Florêncio Figueiredo, José C Alves-Filho, Fernando Q Cunha, Christos Tsatsanis, Christophe Chevillard, Edecio Cunha-Neto, Emilio Hirsch, João S Silva, Thiago M Cunha
Chagas disease is caused by infection with the protozoan Trypanosoma cruzi (T. cruzi) and is an important cause of severe inflammatory heart disease. However, the mechanisms driving Chagas disease cardiomyopathy have not been completely elucidated. Here, we show that the canonical PI3Kγ pathway is upregulated in both human chagasic hearts and hearts of acutely infected mice. PI3Kγ-deficient mice and mutant mice carrying catalytically inactive PI3Kγ are more susceptible to T. cruzi infection. The canonical PI3Kγ signaling in myeloid cells is essential to restrict T...
April 17, 2018: Nature Communications
https://www.readbyqxmd.com/read/29666183/dilated-cardiomyopathy-myosin-mutants-have-reduced-force-generating-capacity
#8
Zoltan Ujfalusi, Carlos D Vera, Srbolujub M Mijailovich, Marina Svicevic, Elizabeth Choe Yu, Masataka Kawana, Kathleen Ruppel, James A Spudich, Michael A Geeves, Leslie A Leinwand
Dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) can cause arrhythmias, heart failure, and cardiac death. Here, we functionally characterized the motor domains of five DCM-causing mutations in human β-cardiac myosin. Kinetic analyses of the individual events in the ATPase cycle revealed that each mutation alters different steps in this cycle. For example, different mutations gave enhanced or reduced rate constants of ATP binding, ATP hydrolysis, or ADP release or exhibited altered ATP, ADP, or actin affinity...
April 17, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29665860/the-use-of-cangrelor-with-heparin-for-left-ventricular-assist-device-implantation-in-a-patient-with-acute-heparin-induced-thrombocytopenia
#9
Yan K Gernhofer, Michael Ross, Swapnil Khoche, Victor Pretorius
BACKGROUND: Optimal anticoagulation strategy for cardiopulmonary bypass (CPB) in end-stage heart failure patients with heparin-induced thrombocytopenia (HIT) requiring left ventricular assist device (LVAD) implantation remains uncertain. Presently, there are no large-scale randomized studies comparing outcomes of alternative anticoagulation strategies for CPB in this patient population. A novel antiplatelet agent - cangrelor, which is a potent P2Y12 inhibitor with robust antiplatelet efficacy, rapid reversibility, and measurable drug effect, has become available since 2015...
April 17, 2018: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29665072/an-update-on-canine-cardiomyopathies-is-it-all-in-the-genes
#10
REVIEW
E Dutton, J López-Alvarez
Dilated cardiomyopathy is the second most common cardiac disease in dogs and causes considerable morbidity and mortality. Primary dilated cardiomyopathy is suspected to be familial, and genetic loci have been associated with the disease in a number of breeds. Because it is an adult-onset disease, usually with late onset, testing breeding dogs and bitches before breeding for a genetic mutation that could lead to dilated cardiomyopathy would be helpful to prevent disease. There is growing evidence that the genetic basis may be multigenic rather than monogenic in the majority of studied breeds...
April 17, 2018: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/29665007/protective-effect-of-mir378-on-doxorubicin-induced-cardiomyocyte-injury-via-calumenin
#11
Yu Wang, Xiaoxue Cui, Yilin Wang, Yao Fu, Xin Guo, Jie Long, Chengxi Wei, Ming Zhao
Doxorubicin (Dox) is a highly effective antitumor antibiotic, however myocardial toxicity severely limits its use clinically. The pathogenesis of doxorubicin-induced cardiomyopathy is unclear. In Dox cardiomyopathy mice, there is a decline in cardiac function, a change in myocardial pathology and a reduction in miR378* expression. Expression changes in calumenin, an endoplasmic reticulum stress (ERS) chaperone protein and pathway factor, as well as apoptosis, were observed in cardiomyocytes after doxorubicin-induced injury...
April 17, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29664407/mechanical-circulatory-support-device-utilization-and-heart-transplant-waitlist-outcomes-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#12
Lakshmi Sridharan, Brian Wayda, Lauren K Truby, Farhana Latif, Susan Restaino, Koji Takeda, Hiroo Takayama, Yoshifumi Naka, Paolo C Colombo, Mathew Maurer, Maryjane A Farr, Veli K Topkara
BACKGROUND: Patients with restrictive cardiomyopathy (RCM) and hypertrophic cardiomyopathy (HCM) generally are considered poor candidates for mechanical circulatory support devices (MCSDs) and often not able to be bridged mechanically to heart transplantation. This study characterized MCSD utilization and transplant waitlist outcomes in patients with RCM/HCM under the current allocation system and discusses changes in the era of the new donor allocation system. METHODS AND RESULTS: Patients waitlisted from 2006 to 2016 in the United Network for Organ Sharing registry were stratified by RCM/HCM versus other diagnoses...
March 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29664404/outcomes-in-patients-with-hypertrophic-cardiomyopathy-awaiting-heart-transplantation
#13
Julio Zuñiga Cisneros, Josef Stehlik, Craig H Selzman, Stavros G Drakos, Stephen H McKellar, Omar Wever-Pinzon
BACKGROUND: Current organ allocation policy and the rapid growth of mechanical support favor heart transplant (HT) candidates on left ventricular assist devices. HT candidates with hypertrophic cardiomyopathy (HCM) are usually not left ventricular assist device candidates and may have a disadvantage compared with dilated forms of cardiomyopathy. METHODS AND RESULTS: Adult HT candidates registered in the Scientific Registry of Transplant Recipients database between 1999 and 2016 were included...
March 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29663894/coenzyme-q10-in-cardiovascular-and-metabolic-diseases-current-state-of-the-problem
#14
Vladlena I Zozina, Serghei Covantev, Olga A Goroshko, Liudmila M Krasnykh, Vladimir G Kukes
The burden of cardiovascular and metabolic diseases is increasing with every year. Although the management of these conditions has improved greatly over the years it is still far from perfect. With all of this in mind, there is a need for new methods of prophylaxis and treatment. Coenzyme Q10 (CoQ10) is an essential compound of the human body. There is growing evidence that CoQ10 is tightly linked to cardiometabolic disorders. Its supplementation can be useful in a variety of chronic and acute disorders. This review analyses the role of CoQ10 in hypertension, ischemic heart disease, myocardial infarction, heart failure, viral myocarditis, cardiomyopathies, cardiac toxicity, dyslipidemia, obesity, type 2 diabetes mellitus, metabolic syndrome, cardiac procedures and resuscitation...
April 15, 2018: Current Cardiology Reviews
https://www.readbyqxmd.com/read/29663861/amyloid-cardiomyopathy-a-hidden-heart-failure-cause-that-is-often-misdiagnosed
#15
Yale Tung-Chen, Miguel-Ángel Arnau
BACKGROUND: Congestive heart failure (CHF) is a very common condition, especially in the elderly, characterized by dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and peripheral edema. CLINICAL PRESENTATION AND INTERVENTION: We report the case of a 76-year-old with CHF symptoms for the last 3 months. The ECG and transthoracic echocardiogram were suggestive of CHF due to amyloid cardiomyopathy. After cardiac MRI, a positive Congo red staining of subcutaneous fat aspiration and a negative genetic testing for mutant transthyretin, senile amyloid cardiomyopathy (ATTRw) was expected...
April 17, 2018: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29663803/simultaneous-fluorescence-visualization-of-endoplasmic-reticulum-superoxide-anion-and-polarity-in-myocardial-cells-and-tissue
#16
Haibin Xiao, Chuanchen Wu, Ping Li, Bo Tang
Diabetic cardiomyopathy (DCM) is a critical complication of diabetes, the accurate pathogenesis of which remains elusive. It's widely accepted that endoplasmic reticulum (ER) stress and abnormal fluctuations of reactive oxygen species (ROS) are considered to be closely associated with progress of DCM. In addition, DCM-induced changes of myocardial tissue and ROS-derived oxidation of proteins will cause changes of the hydrophilic and hydrophobic domains and may further seriously alter the myocardial cell polarity...
April 17, 2018: Analytical Chemistry
https://www.readbyqxmd.com/read/29663722/homozygous-missense-mybpc3-pro873his-mutation-associated-with-increased-risk-for-heart-failure-development-in-hypertrophic-cardiomyopathy
#17
Antheia Kissopoulou, Cecilia Trinks, Anna Green, Jan-Erik Karlsson, Jon Jonasson, Cecilia Gunnarsson
Hypertrophic cardiomyopathy (HCM) is a primary autosomal-dominant disorder of the myocardium with variable expressivity and penetrance. Occasionally, homozygous sarcomere genetic variants emerge while genotyping HCM patients. In these cases, a more severe HCM phenotype is generally seen. Here, we report a case of HCM that was diagnosed clinically at 39 years of age. Initial symptoms were shortness of breath during exertion. Successively, he developed a wide array of severe clinical manifestations, which progressed to an ominous end-stage heart failure that resulted in heart transplantation...
April 16, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29663451/association-of-prolonged-qtc-interval-with-takotsubo-cardiomyopathy-a-neurocardiac-syndrome-inside-the-mystery-of-the-insula-of-reil
#18
REVIEW
Vincenzo Marafioti, Giulia Turri, Vincenzo Carbone, Salvatore Monaco
The Takotsubo cardiomyopathy is often considered autochthonous to the heart, although the primary problem may be not in the heart muscle itself. Instead, similar to several Takotsubo-like cardiac pathologies seen in acute neurological diseases, it may reflect the capacity of the nervous system to injure the heart. Persuasive evidence exists that shocking emotional stress promotes direct heart injuries. Moreover, clinical and laboratory research shows that cardiac structural damage can occur in the presence of a normal heart, especially in the context of seizures, stroke, and traumatic brain injury or under conditions of psychological stress...
April 17, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29663442/complexity-of-scar-and-ventricular-arrhythmias-in-dilated-cardiomyopathy-of-any-etiology-long-term-data-from-the-scarfear-cardiovascular-magnetic-resonance-predictors-of-appropriate-implantable-cardioverter-defibrillator-therapy-delivery-registry
#19
Stefano Pedretti, Sara Vargiu, Matteo Baroni, Santo Dellegrottaglie, Barbara Lanzarin, Alberto Roghi, Angela Milazzo, Giuseppina Quattrocchi, Maurizio Lunati, Patrizia Pedrotti
BACKGROUND: Late gadolinium enhancement (LGE) assessed with cardiovascular magnetic resonance (CMR) correlates with ventricular arrhythmias and survival in patients with structural heart disease. Whether some LGE characteristics may specifically improve prediction of arrhythmic outcomes is unknown. HYPOTHESIS: We sought to evaluate scar characteristics assessed with CMR to predict implantable cardioverter-defibrillator (ICD) interventions in dilated cardiomyopathy of different etiology...
April 17, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29663123/creation-of-a-restrictive-atrial-communication-in-heart-failure-with-preserved-and-mid-range-ejection-fraction-effective-palliation-of-left-atrial-hypertension-and-pulmonary-congestion
#20
Anna Bauer, Markus Khalil, Monika Lüdemann, Jürgen Bauer, Anoosh Esmaeili, Roberta De-Rosa, Norbert F Voelkel, Hakan Akintuerk, Dietmar Schranz
BACKGROUND: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. METHODS AND RESULTS: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %...
April 16, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
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