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restrictive cardiomyopathy

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https://www.readbyqxmd.com/read/29777282/ambulatory-intravenous-inotropic-support-and-or-levosimendan-in-pediatric-and-congenital-heart-failure-safety-survival-improvement-or-transplantation
#1
Sotiria C Apostolopoulou, George A Vagenakis, Alexandros Tsoutsinos, Felicia Kakava, Spyridon Rammos
End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29754565/il-27-genetic-variation-and-susceptibility-of-dilated-cardiomyopathy-in-chinese-han-population
#2
Yu Chen, Rui Zhang, Linjun Zeng, Hong Wei, Yang Chen, Jianhui Zeng
AIM: Accumulating data showed that IL-27 polymorphisms are linked to the susceptibility of some autoimmune diseases. We assessed whether there was an association between two single nucleotide polymorphisms (SNPs) of IL-27 gene and dilated cardiomyopathy (DCM). MATERIALS & METHODS: Two SNPs (rs153109 and rs17855750) of IL-27 gene were genotyped by PCR-restriction fragment length polymorphism in 261 DCM patients and 303 unrelated healthy subjects in Chinese Han population...
September 2017: Personalized Medicine
https://www.readbyqxmd.com/read/29752569/widespread-expression-of-perilipin-5-in-normal-human-tissues-and-in-diseases-is-restricted-to-distinct-lipid-droplet-subpopulations
#3
Merita Hashani, Hagen Roland Witzel, Lena Maria Pawella, Judith Lehmann-Koch, Jens Schumacher, Gunhild Mechtersheimer, Martina Schnölzer, Peter Schirmacher, Wilfried Roth, Beate Katharina Straub
Diseases associated with the accumulation of lipid droplets are increasing in western countries. Lipid droplet biogenesis, structure and degradation are regulated by proteins of the perilipin family. Perilipin 5 has been shown to regulate basal lipolysis in oxidative tissues. We examine perilipin 5 in normal human tissues and in diseases using protein biochemical and microscopic techniques. Perilipin 5 was constitutively located at small lipid droplets in skeletal myocytes, cardiomyocytes and brown adipocytes...
May 11, 2018: Cell and Tissue Research
https://www.readbyqxmd.com/read/29741282/restrictive-cardiomyopathy-an-unusual-phenotype-of-a-lamin-a-variant
#4
Mark S Paller, Cindy M Martin, Mary Ella Pierpont
Most individuals with cardiomyopathy associated with variants of the LMNA (lamin A) gene present with cardiac conduction abnormalities followed by dilated cardiomyopathy and cardiac failure; some also have skeletal muscle weakness. In this report, an individual with restrictive cardiomyopathy presenting with conduction defects followed by cardiac dysfunction of a restrictive nature eventually requiring cardiac transplantation is described. Subsequently, progressive skeletal muscle weakness became evident. The finding of a new LMNA pathologic gene variant in this patient increases the options for genetic testing of individuals with restrictive cardiomyopathy...
May 9, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29729610/brain-death-organ-donor-supported-by-a-left-ventricular-assist-device-showing-unexpected-congestive-liver-fibrosis-a-case-report
#5
Hideya Kamei, Masahiko Komagome, Nobuhiko Kurata, Satoshi Ogiso, Yasuharu Onishi, Takanobu Hara, Mitsuhisa Takatsuki, Susumu Eguchi, Yasuhiro Ogura
INSTRUCTION: Organ transplantation from a brain death donor on mechanical circulatory support is rare. We report a case in which a brain death donor, supported by a left ventricular assist device (LVAD), unexpectedly displayed significant congestive fibrosis of the liver. PRESENTATION OF CASE: The potential organ donor was diagnosed 23 years previously as having dilated-phase of hypertrophic cardiomyopathy. He had undergone implantation of an LVAD as a bridge to heart transplantation...
April 30, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29716714/evaluation-of-a-polymorphism-in-mybpc3-in-patients-with-anthracycline-induced-cardiotoxicity
#6
M Thirumalai Vinodhini, S Sneha, R P Nagare, S Bindhya, V Shetty, D Manikandan, P Ganesan, T G Sagar, T S Ganesan
Cardiotoxicity is the most serious side effect of anthracyclines (doxorubicin, daunorubicin or epirubicin). The incidence of anthracycline induced late cardiac toxicity (AIC) that is overt clinically is 3-5% in the Indian population. Polymorphism in intron 32 (deletion of 25bp) of MYBPC3 has been shown to be present exclusively in Asians and more so in South India (3-8%). The frequency of the polymorphism is significantly higher (13%) in patients with cardiomyopathy in India. Fifteen patients were identified to have cardiac dysfunction following treatment for malignant lymphoma with doxorubicin containing regimens...
March 2018: Indian Heart Journal
https://www.readbyqxmd.com/read/29701428/anaesthetic-management-in-a-duchennne-muscle-dystrophy-patient-for-treatment-of-recurrent-pneumothorax
#7
Sara Mota, Liuba Germanova, Joana Cortesão, Teresa Paiva
INTRODUCTION: Duchenne muscular dystrophy (DMD) is an x linked recessive disorder. Long term prognosis is ominous, with development of respiratory distress and cardiomyopathy in advanced stage of the disease and expected death in the teens-to-mid 20s due to respiratory or cardiac failure. Peri-operative management of this patients is challenging due to difficult airway anatomy (macroglossia, limited neck and mandibular mobility). Additionally, they are at risk of developing malignant hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anaesthetics and depolarizing muscle relaxants...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
https://www.readbyqxmd.com/read/29700090/cardiac-amyloidosis
#8
Ana Martinez-Naharro, Philip N Hawkins, Marianna Fontana
Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis...
April 1, 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29688316/diagnostic-and-therapeutic-strategies-for-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-patient
#9
Weijia Wang, Cynthia A James, Hugh Calkins
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare inherited heart muscle disease characterized by ventricular tachyarrhythmia, predominant right ventricular dysfunction, and sudden cardiac death. Its pathophysiology involves close interaction between genetic mutations and exposure to physical activity. Mutations in genes encoding desmosomal protein are the most common genetic basis. Genetic testing plays important roles in diagnosis and screening of family members. Syncope, palpitation, and lightheadedness are the most common symptoms...
April 23, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29686627/burst-like-transcription-of-mutant-and-wildtype-myh7-alleles-as-possible-origin-of-cell-to-cell-contractile-imbalance-in-hypertrophic-cardiomyopathy
#10
Judith Montag, Kathrin Kowalski, Mirza Makul, Pia Ernstberger, Ante Radocaj, Julia Beck, Edgar Becker, Snigdha Tripathi, Britta Keyser, Christian Mühlfeld, Kirsten Wissel, Andreas Pich, Jolanda van der Velden, Cristobal G Dos Remedios, Andreas Perrot, Antonio Francino, Francesco Navarro-López, Bernhard Brenner, Theresia Kraft
Hypertrophic Cardiomyopathy (HCM) has been related to many different mutations in more than 20 different, mostly sarcomeric proteins. While development of the HCM-phenotype is thought to be triggered by the different mutations, a common mechanism remains elusive. Studying missense-mutations in the ventricular beta-myosin heavy chain (β-MyHC, MYH7 ) we hypothesized that significant contractile heterogeneity exists among individual cardiomyocytes of HCM-patients that results from cell-to-cell variation in relative expression of mutated vs...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29681957/mechanical-circulatory-support-is-effective-to-treat-pulmonary-hypertension-in-heart-transplant-candidates-disqualified-due-to-unacceptable-pulmonary-vascular-resistance
#11
Michał Zakliczyński, Jerzy Pacholewicz, Izabela Copik, Marcin Maruszewski, Tomasz Hrapkowicz, Roman Przybylski, Marian Zembala
Introduction: High pulmonary vascular resistance (PVR) in orthotopic heart transplantation (OHT) candidates is a risk factor of right ventricle failure after the procedure. However, the increase of PVR may be a consequence of the life-threatening deterioration of the left ventricle function. The use of mechanical circulatory support (MCS) seems to be the best solution, but it is reimbursed only in active OHT candidates. Aim: We performed a retrospective analysis of MCS effectiveness in maintaining PVR at values accepted for OHT...
March 2018: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/29679024/cysteine-protease-cathepsins-in-cardiovascular-disease-from-basic-research-to-clinical-trials
#12
REVIEW
Cong-Lin Liu, Junli Guo, Xian Zhang, Galina K Sukhova, Peter Libby, Guo-Ping Shi
Cysteine protease cathepsins have traditionally been considered as lysosome-restricted proteases that mediate proteolysis of unwanted proteins. However, studies from the past decade demonstrate that these proteases are localized not only in acidic compartments (endosomes and lysosomes), where they participate in intracellular protein degradation, but also in the extracellular milieu, plasma membrane, cytosol, nucleus, and nuclear membrane, where they mediate extracellular matrix protein degradation, cell signalling, and protein processing and trafficking through the plasma and nuclear membranes and between intracellular organelles...
April 20, 2018: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/29666415/canonical-pi3k%C3%AE-signaling-in-myeloid-cells-restricts-trypanosoma-cruzi-infection-and-dampens-chagasic-myocarditis
#13
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/29664407/mechanical-circulatory-support-device-utilization-and-heart-transplant-waitlist-outcomes-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#14
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/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
#15
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
https://www.readbyqxmd.com/read/29659121/glucagon-like-peptide-1-ameliorates-cardiac-lipotoxicity-in-diabetic-cardiomyopathy-via-the-ppar%C3%AE-pathway
#16
Lujin Wu, Ke Wang, Wei Wang, Zheng Wen, Peihua Wang, Lei Liu, Dao Wen Wang
Lipotoxicity cardiomyopathy is the result of excessive accumulation and oxidation of toxic lipids in the heart. It is a major threat to patients with diabetes. Glucagon-like peptide-1 (GLP-1) has aroused considerable interest as a novel therapeutic target for diabetes mellitus because it stimulates insulin secretion. Here, we investigated the effects and mechanisms of the GLP-1 analog exendin-4 and the dipeptidyl peptidase-4 inhibitor saxagliptin on cardiac lipid metabolism in diabetic mice (DM). The increased myocardial lipid accumulation, oxidative stress, apoptosis, and cardiac remodeling and dysfunction induced in DM by low streptozotocin doses and high-fat diets were significantly reversed by exendin-4 and saxagliptin treatments for 8 weeks...
April 16, 2018: Aging Cell
https://www.readbyqxmd.com/read/29607544/outcome-of-pediatric-heart-transplantation-in-blood-culture-positive-donors-in-the-united-states
#17
Shahnawaz M Amdani, Wei Du, Sanjeev Aggarwal
Active donor infection at time of organ procurement poses a potential infection risk and may increase post-transplant morbidity and mortality in recipients. Our hypothesis was that pediatric heart transplant recipients from blood culture positive donors (BCPD) would have increased morbidity and mortality compared to non-blood culture positive donors (NBCPD). A retrospective analyses of pediatric heart transplant recipients using the Organ Procurement and Transplant Network (OPTN) between 1987 and 2015 were conducted...
April 1, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29603321/incremental-value-of-the-tissue-motion-of-annular-displacement-derived-from-speckle-tracking-echocardiography-for-differentiating-chronic-constrictive-pericarditis-from-restrictive-cardiomyopathy
#18
Shuang Liu, Weidong Ren, Jing Zhang, Chunyan Ma, Jun Yang, Yan Zhang, Zhengyu Guan
OBJECTIVES: The tissue motion of annular displacement provides an accurate and rapid assessment of left ventricular (LV) systolic function. However, it has rarely been used in patients with chronic constrictive pericarditis and restrictive cardiomyopathy. This study aimed to assess the differences in LV systolic function in patients with constrictive pericarditis and restrictive cardiomyopathy using tissue motion of annular displacement derived from speckle-tracking echocardiography. METHODS: Twenty-four patients with constrictive pericarditis, 24 with restrictive cardiomyopathy, and 25 healthy volunteers (controls) were enrolled...
March 30, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29602442/-tropical-endomyocardial-fibrosis-perspectives
#19
M Lachaud, C Lachaud, D Sidi, A Menete, X Jouven, E Marijon, B Ferreira
Tropical endomyocardial fibrosis (FET) is a leading cause of heart failure and the most common restrictive cardiomyopathy worldwide. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling and valvular insufficiency define the disease. Confined to peculiar and limited geographical areas, the aetiology remains blurred and it carries a grim prognosis. The changing burden reported recently in some endemic areas and the refinement of diagnostic tools have emphasized alternative routes for understanding and treatment of the disease...
March 27, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29579879/short-term-use-of-ventricular-assist-device-in-a-patient-with-restrictive-cardiomyopathy-a-case-report
#20
F Rodríguez-Mora, E M Cantero-Pérez, C A Rojas-Bermúdez, J M Borrego-Domínguez, Á Herruzo-Avilés, R Hinojosa-Pérez, A Adsuar-Gómez
The CentriMag (Levitronix LLC, Waltham, MA, USA) ventricular assist device is a centrifugal pump designed for short-term support in patients with cardiogenic shock. In patients with restrictive physiology, there is some concern about the use of these devices due a very small ventricular cavity. We review the evolution of a 32-year-old woman with restrictive cardiomyopathy, moderate pulmonary hypertension, and severe biventricular systo-diastolic dysfunction in whom a biventricular Centrimag device was implanted due to a progressive clinical worsening...
March 2018: Transplantation Proceedings
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