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restrictive cardiomyopathy

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https://www.readbyqxmd.com/read/29078120/adventitial-tertiary-lymphoid-organ-classification-in-human-atherosclerosis
#1
Mohammadreza Akhavanpoor, Christian A Gleissner, Hamidreza Akhavanpoor, Felix Lasitschka, Andreas O Doesch, Hugo A Katus, Christian Erbel
BACKGROUND: Atherosclerosis is a chronic inflammatory disease of the arterial wall. Adjacent to lamina intima lesion progression, a cellular compound develops in the lamina adventitia, defined as tertiary lymphoid organs (TLO) in mice. But in human system, it remains unknown whether these adventitial cellular accumulations represent these highly organized immunological structures. PATIENTS AND METHODS: In this study, we investigated whether the adventitial cellular compounds represent TLOs in 72 human coronary artery samples by immunoenzyme staining...
September 7, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/29071521/cmr-assessment-and-clinical-outcomes-of-hypertrophic-cardiomyopathy-with-or-without-ventricular-remodeling-in-the-end-stage-phase
#2
Sainan Cheng, Yeon Hyeon Choe, Hideki Ota, Chen Cui, Gang Yin, Minjie Lu, Lu Li, Xiuyu Chen, Sanjay K Prasad, Shihua Zhao
End-stage phase of hypertrophic cardiomyopathy (ES-HCM) is a recognized part of HCM disease spectrum. Information on cardiac magnetic resonance (CMR) studies for ES-HCM especially for those without ventricular remodeling has been limited. We aimed to evaluate the morpho-functional and tissue features of ES-HCM with or without ventricular remodeling and to explore CMR prognostic value in these patients. We analysed CMR scans of sixty-three ES-HCM patients and divided them into those with ventricular dilatation (D-ES, n = 41) and those with normal ventricular size (N-ES, n = 22)...
October 25, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29068766/the-role-of-autophagy-in-the-heart
#3
Sebastiano Sciarretta, Yasuhiro Maejima, Daniela Zablocki, Junichi Sadoshima
Autophagy is an evolutionarily conserved mechanism by which cytoplasmic elements are degraded intracellularly. Autophagy has also emerged as a major regulator of cardiac homeostasis and function. Autophagy preserves cardiac structure and function under baseline conditions and is activated during stress, limiting damage under most conditions. It reduces injury and preserves cardiac function during ischemia. It also reduces chronic ischemic remodeling and mediates the cardiac adaptation to pressure overload by restricting misfolded protein accumulation, mitochondrial dysfunction, and oxidative stress...
October 25, 2017: Annual Review of Physiology
https://www.readbyqxmd.com/read/29067653/clinical-manifestations-and-overall-management-strategies-for-duchenne-muscular-dystrophy
#4
Takeshi Tsuda
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder that causes progressive weakness and wasting of skeletal muscular and myocardium in boys due to mutation of dystrophin. The structural integrity of each individual skeletal and cardiac myocyte is significantly compromised upon physical stress due to the absence of dystrophin. The progressive destruction of systemic musculature and myocardium causes affected patients to develop multiple organ disabilities, including loss of ambulation, physical immobility, neuromuscular scoliosis, joint contracture, restrictive lung disease, obstructive sleep apnea, and cardiomyopathy...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29047356/non-syndromic-cardiac-progeria-in-a-patient-with-the-rare-pathogenic-p-asp300asn-variant-in-the-lmna-gene
#5
Ali J Marian
BACKGROUND: Mutations in LMNA gene, encoding Lamin A/C, cause a diverse array of phenotypes, collectively referred to as laminopathies. The most common manifestation is dilated cardiomyopathy (DCM), occurring in conjunction with variable skeletal muscle involvement but without involvement of the coronary arteries. Much less commonly, LMNA mutations cause progeroid syndromes, whereby an early-onset coronary artery disease (CAD) is the hallmark of the disease. We report a hitherto unreported compound cardiac phenotype, dubbed as "non-syndromic cardiac progeria", in a young patient who carried a rare pathogenic variant in the LMNA gene and developed progressive degeneration of various cardiac structures, as seen in the elderly...
October 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29046207/-clinical-characteristics-and-gaa-gene-mutation-in-children-with-glycogen-storage-disease-type-ii-an-analysis-of-3-cases
#6
Shan Yuan, Jie Jiang, Lu-Ting Zha, Zuo-Cheng Yang
Glycogen storage disease type II (GSD II) is an autosomal recessive disorder caused by a deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase (GAA) and can affect multiple systems including the heart and skeletal muscle. The aim of this study was to investigate three children with GSD II confirmed by GAA gene analysis and to report their clinical characteristics and gene mutations. One case was classified as infantile-onset GSD II, and two cases as late-onset GSD II. The infantile-onset patient (aged 4 months) showed no weight increase and had dyspnea, muscle hypotonia, and increased alanine aminotransferase and creatine kinase; echocardiography showed hypertrophic cardiomyopathy...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29025547/contemporary-techniques-of-pericardiectomy-for-pericardial-disease
#7
REVIEW
Pouya Hemmati, Kevin L Greason, Hartzell V Schaff
Pericardiectomy is a potentially curative treatment for constrictive pericarditis. We use a median sternotomy and believe that adequate resection involves removal of the diaphragmatic pericardium and the anterior pericardium. Late outcomes depend on severity of right-sided heart failure preoperatively, the etiology of constrictive pericarditis, and adequate pericardial resection. Late results are excellent in patients with idiopathic disease or those with pericarditis secondary to prior cardiac operations. However, survival is reduced in those with radiation-induced constrictive pericarditis, primarily owing to additional secondary effects of radiation on cardiac valves, epicardial coronary arteries, and ventricular myocardium where fibrosis may cause associated restrictive cardiomyopathy...
November 2017: Cardiology Clinics
https://www.readbyqxmd.com/read/29024504/high-validity-of-cardiomyopathy-diagnoses-in-western-sweden-1989-2009
#8
Carmen Basic, Annika Rosengren, Sandra Lindström, Maria Schaufelberger
AIM: Hospital discharges with a diagnosis of cardiomyopathy have more than doubled in Sweden since 1987. We validated the cardiomyopathy diagnoses over this time period to investigate that the increase was real and not a result of improved recognition of the diagnosis and better diagnostic methods. METHODS AND RESULTS: Every fifth year from 1989 to 2009, records for all patients with a cardiomyopathy diagnosis were identified by searching the local registers in three hospitals in Västra Götaland, Sweden...
October 11, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/29021349/activation-of-autophagy-ameliorates-cardiomyopathy-in-mybpc3-targeted-knockin-mice
#9
Sonia R Singh, Antonia T L Zech, Birgit Geertz, Silke Reischmann-Düsener, Hanna Osinska, Maksymilian Prondzynski, Elisabeth Krämer, Qinghang Meng, Charles Redwood, Jolanda van der Velden, Jeffrey Robbins, Saskia Schlossarek, Lucie Carrier
BACKGROUND: Alterations in autophagy have been reported in hypertrophic cardiomyopathy (HCM) caused by Danon disease, Vici syndrome, or LEOPARD syndrome, but not in HCM caused by mutations in genes encoding sarcomeric proteins, which account for most of HCM cases. MYBPC3, encoding cMyBP-C (cardiac myosin-binding protein C), is the most frequently mutated HCM gene. METHODS AND RESULTS: We evaluated autophagy in patients with HCM carrying MYBPC3 mutations and in a Mybpc3-targeted knockin HCM mouse model, as well as the effect of autophagy modulators on the development of cardiomyopathy in knockin mice...
October 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28992800/antimalarial-induced-cardiomyopathy-a-systematic-review-of-the-literature
#10
K Tselios, M Deeb, D D Gladman, P Harvey, M B Urowitz
Background Antimalarials (AMs) are widely used in the treatment of connective tissue diseases. Their main side effect is retinal damage, while heart disease has been described in isolated cases. The aim of this study is to systematically review the existing literature on AM-induced cardiomyopathy (AMIC). Methods The PubMed database was searched for heart biopsy-confirmed AMIC cases. Information on demographics, clinical presentation, concomitant AM-related toxicity, cardiological investigations, treatment and outcome were collected...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28986453/reduced-cardiac-calcineurin-expression-mimics-long-term-hypoxia-induced-heart-defects-in-drosophila
#11
Rachel Zarndt, Stanley M Walls, Karen Ocorr, Rolf Bodmer
BACKGROUND: Hypoxia is often associated with cardiopulmonary diseases, which represent some of the leading causes of mortality worldwide. Long-term hypoxia exposures, whether from disease or environmental condition, can cause cardiomyopathy and lead to heart failure. Indeed, hypoxia-induced heart failure is a hallmark feature of chronic mountain sickness in maladapted populations living at high altitude. In a previously established Drosophila heart model for long-term hypoxia exposure, we found that hypoxia caused heart dysfunction...
October 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28979560/a-case-study-of-likely-wild-type-cardiac-transthyretin-amyloidosis-causing-rapid-deterioration
#12
Thomas Davies, Aarash Saleh, Gerry Coghlan, Carol Whelan, Banwari Agarwal
We present the case of an 88-year-old gentleman who presented to hospital septic with bilateral leg cellulitis, pulmonary oedema and hypotension. He had no history of heart disease but had had bilateral carpal tunnel releases. His condition deteriorated with refractory hypotension in spite of fluid filling, inotropic and vasopressor support. His echocardiogram showed an infiltrative cardiomyopathy with a speckled myocardium, severe concentric left and right ventricular increased wall thickness, diastolic dysfunction, biatrial dilatation and restrictive physiology in keeping with cardiac amyloidosis...
May 2017: J Intensive Care Soc
https://www.readbyqxmd.com/read/28970073/outcomes-of-restrictive-and-hypertrophic-cardiomyopathies-after-lvad-an-intermacs-analysis
#13
Snehal R Patel, Omar Saeed, David Naftel, Susan Myers, James Kirklin, Ulrich P Jorde, Daniel J Goldstein
BACKGROUND: Patients with restrictive (RCM) and hypertrophic (HCM) cardiomyopathies are felt to be a difficult population to treat with left ventricular assist device (LVAD) therapy. Scarce data exist on outcomes of continuous-flow (CF) LVAD support in these challenging patient cohorts. METHODS: The Interagency Registry for Mechanically Assisted Circulatory Support Registry was queried for all patients with RCM (n = 94) and HCM (n = 104) who underwent CF LVAD implantation between March 2008 and March 2014...
September 29, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28964914/imatinib-mesylate-induced-cardiomyopathy-involves-resident-cardiac-progenitors
#14
Monia Savi, Caterina Frati, Stefano Cavalli, Gallia Graiani, Serena Galati, Annamaria Buschini, Denise Madeddu, Angela Falco, Lucia Prezioso, Giulia Mazzaschi, Federica Galaverna, Costanza Anna Maria Lagrasta, Emilia Corradini, Antonella De Angelis, Donato Cappetta, Liberato Berrino, Franco Aversa, Federico Quaini, Konrad Urbanek
Cardiovascular complications are included among the systemic effects of tyrosine kinase inhibitor (TKI)-based therapeutic strategies. To test the hypothesis that inhibition of Kit tyrosine kinase that promotes cardiac progenitor cell (CPC) survival and function may be one of the triggering mechanisms of imatinib mesylate (IM)-related cardiovascular effects, the anatomical, structural and ultrastructural changes in the heart of IM-treated rats were evaluated. Cardiac anatomy in IM-exposed rats showed a dose-dependent, restrictive type of remodeling and depressed hemodynamic performance in the absence of remarkable myocardial fibrosis...
September 27, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28964475/pediatric-cardiac-transplantation
#15
Thomas D Ryan, Clifford Chin
Heart transplantation in pediatric patients generally arises as a treatment option of last resort, that is, the indication is for patients with heart failure of various etiologies, with potential or actual end-organ dysfunction, in whom there are no reasonable, long-term options for life-prolonging therapy. The concept of heart failure is complex in a pediatric population, particularly those with congenital heart disease. While heart failure may refer simply to systolic dysfunction leading to low cardiac output, it can also encompass: diastolic dysfunction in restrictive cardiomyopathy; single ventricle physiology without an option for stable palliation...
August 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28957535/exercise-and-the-right-ventricle-a-potential-achilles-heel
#16
Andre La Gerche, Dhrubo J Rakhit, Guido Claessen
Exercise is associated with unequivocal health benefits and results in many structural and functional changes of the myocardium that enhance performance and prevent heart failure. However, intense exercise also presents a significant hemodynamic challenge in which the right-sided heart chambers are exposed to a disproportionate increase in afterload and wall stress that can manifest as myocardial fatigue or even damage if intense exercise is sustained for prolonged periods. This review focuses on the physiological factors that result in a disproportionate load on the right ventricle during exercise and the long-term consequences...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28951660/treatment-with-tafamidis-slows-disease-progression-in-early-stage-transthyretin-cardiomyopathy
#17
Marla B Sultan, Balarama Gundapaneni, Jennifer Schumacher, Jeffrey H Schwartz
BACKGROUND: Transthyretin cardiomyopathy (TTR-CM) is a progressive, fatal disease caused by the accumulation of misfolded transthyretin (TTR) amyloid fibrils in the heart. Tafamidis is a kinetic stabilizer of TTR that inhibits misfolding and amyloid formation. METHODS: In this post hoc analysis, data from an observational study (Transthyretin Amyloidosis Cardiac Study; n = 29) were compared with an open-label study of tafamidis in patients with TTR-CM (Fx1B-201; n = 35)...
2017: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/28944178/alcohol-septal-ablation-in-which-patients-and-why
#18
Paolo Spirito, Jessica Rossi, Barry J Maron
At present, surgical septal myectomy is regarded as the "gold standard" treatment for most patients with obstructive hypertrophic cardiomyopathy (HCM) and drug-refractory symptoms. However, the best results are obtained by those surgeons who have extensive experience with this operation at a small number of referral centers. In the mid-1990s, percutaneous alcohol septal ablation was introduced as an alternative to myectomy to reduce LV outflow gradient and heart failure symptoms in patients with obstructive HCM...
July 2017: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28933176/-myocarditis-and-inflammatory-cardiomyopathy
#19
Dalibor Mlejnek, Jan Krejčí
Myocarditis is an inflammation of the heart muscle. The most common cause of myocarditis is viral infection in industrialized countries. Myocarditis with left ventricular dysfunction is called inflammatory cardiomyopathy and is the major cause of dilated cardiomyopathy. The clinical picture is very diverse, most often the patient present with signs of heart failure, arrhythmic symptoms and chest pains. Despite significant advances in non-invasive diagnostics, particularly magnetic resonance imaging, endomyocardial biopsy remains the gold standard of myocarditis diagnosis...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#20
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
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