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restrictive cardiomyopathy

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https://www.readbyqxmd.com/read/28203206/mild-and-short-term-caloric-restriction-prevents-obesity-induced-cardiomyopathy-in-young-zucker-rats-without-changing-in-metabolites-and-fatty-acids-cardiac-profile
#1
Gema Ruiz-Hurtado, Concha F García-Prieto, Helena Pulido-Olmo, Juan P Velasco-Martín, Palmira Villa-Valverde, María E Fernández-Valle, Lisardo Boscá, María Fernández-Velasco, Javier Regadera, Beatriz Somoza, María S Fernández-Alfonso
Caloric restriction (CR) ameliorates cardiac dysfunction associated with obesity. However, most of the studies have been performed under severe CR (30-65% caloric intake decrease) for several months or even years in aged animals. Here, we investigated whether mild (20% food intake reduction) and short-term (2-weeks) CR prevented the obese cardiomyopathy phenotype and improved the metabolic profile of young (14 weeks of age) genetically obese Zucker fa/fa rats. Heart weight (HW) and HW/tibia length ratio was significantly lower in fa/fa rats after 2 weeks of CR than in counterparts fed ad libitum...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28192468/matrix-metalloproteinases-are-required-for-membrane-motility-and-lumenogenesis-during-drosophila-heart-development
#2
Qanber S Raza, Jessica L Vanderploeg, J Roger Jacobs
Matrix Metalloproteinases (Mmps) degrade glycoproteins and proteoglycans of the extracellular matrix (ECM) or cell surface and are crucial for morphogenesis. Mmps and their inhibitors are expressed during early stages of cardiac development in vertebrates and expression is altered in multiple congenital cardiomyopathies such as cardia bifida. Drosophila genome encodes two copies of Mmps, Mmp1 and Mmp2 whereas in humans up to 25 Mmps have been identified with overlapping functions. We investigated the role of Mmps during embryonic heart development in Drosophila, a process which is morphogenetically similar to early heart tube formation in vertebrates...
2017: PloS One
https://www.readbyqxmd.com/read/28174160/novel-dominant-negative-mutation-in-cardiac-troponin-i-causes-severe-restrictive-cardiomyopathy
#3
Saumya Shah, Haran Yogasundaram, Ratnadeep Basu, Faqi Wang, David I Paterson, Tero-Pekka Alastalo, Gavin Y Oudit
No abstract text is available yet for this article.
February 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28163147/senile-cardiac-amyloidosis-clinical-manifestations-and-non-invasive-diagnostic-approach
#4
Dimitrios Varvarousis, Kali Polytarchou, Nikolaos Daskalopoulos, Ioannis Mantas
Cardiac amyloidosis (CA) refers to an uncommon storage disease leading to restrictive cardiomyopathy and conduction abnormalities. Survival is generally poor but depends strongly on the amyloid type. Wild type transthyretin amyloidosis or senile cardiac amyloidosis (SCA) is a disease of the >60-year age group, has a relatively better prognosis among the various amyloid types and is considered to be underdiagnosed. We describe a case of a patient with clinical signs and symptoms of restrictive cardiomyopathy and a suggested diagnosis of SCA and discuss the diagnostic and therapeutic approach...
February 2, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28153106/isolated-subepicardial-right-ventricular-outflow-tract-scar-in-athletes-with-ventricular%C3%A2-tachycardia
#5
Jeroen Venlet, Sebastiaan R D Piers, Jan D H Jongbloed, Alexander F A Androulakis, Yoshihisa Naruse, Dennis W den Uijl, Gijsbert F L Kapel, Marta de Riva, J Peter van Tintelen, Daniela Q C M Barge-Schaapveld, Martin J Schalij, Katja Zeppenfeld
BACKGROUND: High-level endurance training has been associated with right ventricular pathological remodeling and ventricular tachycardia (VT). Although overlap with arrhythmogenic right ventricular cardiomyopathy (ARVC) has been suggested, the arrhythmogenic substrate for VTs in athletes is unknown. OBJECTIVES: The goal of this study was to evaluate whether electroanatomic scar patterns related to sustained VT can distinguish exercise-induced arrhythmogenic remodeling from ARVC and post-inflammatory cardiomyopathies...
February 7, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28148333/ethical-considerations-of-transparency-informed-consent-and-nudging-in-a-patient-with-paediatric-aortic-stenosis-and-symptomatic-left-ventricular-endocardial-fibroelastosis
#6
Constantine D Mavroudis, Thomas Cook, Jeffrey P Jacobs, Constantine Mavroudis
A 9-year-old boy who was born with bicuspid aortic stenosis underwent two unsuccessful aortic valvuloplasty interventions, and by 2 years of age he developed restrictive cardiomyopathy caused by left ventricular endocardial fibroelastosis and diastolic dysfunction. The attending cardiologist referred the patient to a high-volume, high-profile congenital cardiac surgical programme 1000 miles away that has a team with considerable experience with left ventricular endocardial fibroelastosis resection and a reputation of achieving good results...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28120210/renin-angiotensin-system-gene-polymorphisms-as-potential-modifiers-of-hypertrophic-and-dilated-cardiomyopathy-phenotypes
#7
Bindu Rani, Amit Kumar, Ajay Bahl, Rajni Sharma, Rishikesh Prasad, Madhu Khullar
The renin-angiotensin (RAS) pathway has an important role in the etiology of heart failure and given the importance of RAS as a therapeutic target in various cardiomyopathies, genetic polymorphisms in the RAS genes may modulate the risk and severity of disease in cardiomyopathy patients. In the present study, we examined the association of RAS pathway gene polymorphisms, angiotensin converting enzyme (ACE), angiotensinogen (AGT), and angiotensin receptor type 1 (AGTR1) with risk and disease severity in Asian Indian idiopathic cardiomyopathy patients...
March 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28117074/light-chain-cardiac-amyloidosis
#8
REVIEW
Anit K Mankad, Isata Sesay, Keyur B Shah
Cardiac amyloidosis is an underrecognized condition, in which delays to diagnosis have great implications on management options, prognosis, and morbidity. Once cardiac tissue is infiltrated by amyloid fibrils, there is a cascade of pathologic changes that can display an array of clinical manifestations, from impaired relaxation of the ventricular myocardium to severe restrictive disease or even progressive systolic heart failure. Management is guided not only by recognizing the subtype of amyloidosis (primary, hereditary, and wild-type transthyretin amyloidosis), but also the clinical stage of the disease...
November 17, 2016: Current Problems in Cancer
https://www.readbyqxmd.com/read/28110572/-cardiomyopathy-and-ion-channel-diseases-registry-the-szeged-cardiogen-registry
#9
Péter Blazsó, Kornél Kákonyi, Tamás Forster, Róbert Sepp
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia)...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28098235/molecular-characterization-of-pediatric-restrictive-cardiomyopathy-from-integrative-genomics
#10
Tara N Rindler, Robert B Hinton, Nathan Salomonis, Stephanie M Ware
Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28087310/clinical-features-and-three-year-outcomes-of-takotsubo-stress-cardiomyopathy-observational-data-from-one-center
#11
Sigita Glaveckaitė, Pranas Šerpytis, Dovilė Pečiūraitė, Roma Puronaitė, Nomeda Valevičienė
OBJECTIVE: The natural history, management, and outcome of Takotsubo (stress) cardiomyopathy (TTC) is not clear. The aim of this study was to investigate clinical features, define prognostic predictors, and assess the clinical course and outcomes of patients with TTC. METHODS: We analyzed 64 patients (52 women) meeting the proposed Mayo Clinic diagnostic criteria for TTC. All patients were treated at Vilnius University Hospital Santariskiu Klinikos from 2001-01-01 to 2014-11-27...
November 16, 2016: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28080919/restrictive-cardiomyopathy-and-secondary-congestive-heart-failure-in-a-mcdowell-s-carpet-python-morelia-spilota-mcdowelli
#12
Lionel Schilliger, Valérie Chetboul, Cécile Damoiseaux, Alexandra Nicolier
Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations...
December 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28072629/the-key-clues-to-reach-the-diagnosis-of-loeffler-endomyocardial-fibrosis-associated-with-eosinophilic-granulomatosis-with-polyangiitis
#13
Enrico Ammirati, Domenico Sirico, Linda Brevetti, Laura Scudiero, Diana Artioli, Patrizia Pedrotti, Maria Frigerio
Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28050425/severe-left-ventricular-endomyocardial-fibrosis-presenting-as-biventricular-failure-in-a-young-adult-a-case-report
#14
Harpreet Singh Sandhu, Sampathkumar Mahadevappa Mahendrakar, Rajebali Ramzanali Pethani, Azizullah Hafizullah Khan, Yunus Shafi Loya
Endomyocardial Fibrosis (EMF) is a form of progressive restrictive cardiomyopathy of unclear aetiology prevalent in areas within 15(0) of equator including coastal areas of Kerala a few decades back. It inflicts young adults and carries a poor prognosis due to limited options for treatment. Fortunately, the incidence of cases is now declining due to improvement in health and hygiene standards. Here, we review the aetiology and pathogenesis of EMF and report a case of a young male from Mumbai (non-endemic area) presenting with progressively worsening breathlessness and signs of heart failure unresponsive to conventional medical treatment...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28031081/restrictive-cardiomyopathy-resulting-from-a-troponin-i-type-3-mutation-in-a-chinese-family
#15
Yan-Ping Ruan, Chao-Xia Lu, Xiao-Yi Zhao, Rui-Juan Liang, Hui Lian, Michael Routledge, Wei Wu, Xue Zhang, Zhong-Jie Fan
Objective To identify the pathogenic variant responsible for restrictive cardiomyopathy (RCM) in a Chinese family.Methods Next generation sequencing was used for detecting the mutation and Results verified by sequencing. We used restriction enzyme digestion to test the mutation in the family members and 200 unrelated normal subjects without any cardiac inherited diseases when the mutation was identified.Results Five individuals died from cardiac diseases, two of whom suffered from sudden cardiac death. Two individuals have suffered from chronic cardiac disorders...
March 20, 2016: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/28028033/interventions-for-preventing-cardiomyopathy-due-to-anthracyclines-a-bayesian-network-meta-analysis
#16
H Abdel-Qadir, G Ong, R Fazelzad, E Amir, D S Lee, P Thavendiranathan, G Tomlinson
BACKGROUND: The relative efficacy of interventions for primary prevention of anthracycline-associated cardiotoxicity is unknown. METHODS: We conducted a systematic review of randomized controlled trials for primary prevention of anthracycline-associated cardiotoxicity in adult cancer patients. We used hierarchal outcome definitions in the following order of priority: (1) composite of heart failure or decline in left ventricular ejection fraction, (2) decline in ejection fraction, or (3) heart failure...
December 26, 2016: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28011759/-end-stage-heart-failure-therapy-potential-lessons-from-congenital-heart-disease-from-pulmonary-artery-banding-and-interatrial-communication-to-parallel-circulation
#17
REVIEW
Dietmar Schranz, Hakan Akintuerk, Norbert F Voelkel
The final therapy of 'end-stage heart failure' is orthotopic heart, lung or heart-lung transplantation. However, these options are not available for many patients worldwide. Therefore, novel therapeutical strategies are needed. Based on pathophysiological insights regarding (1) the long-term impact of an obstructive pulmonary outflow tract in neonates with congenitally corrected transposition of the great arteries, (2) the importance of a restrictive versus a non-restrictive atrial septum in neonates born with a borderline left ventricle and (3) the significance of both, a patent foramen ovale and/or open ductus arteriosus for survival of newborns with persistent pulmonary hypertension, the current review introduces some therapeutical strategies that may be applicable to selected patients with heart failure...
February 15, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28007147/clinical-and-mechanistic-insights-into-the%C3%A2-genetics-of-cardiomyopathy
#18
REVIEW
Michael A Burke, Stuart A Cook, Jonathan G Seidman, Christine E Seidman
Over the last quarter-century, there has been tremendous progress in genetics research that has defined molecular causes for cardiomyopathies. More than a thousand mutations have been identified in many genes with varying ontologies, therein indicating the diverse molecules and pathways that cause hypertrophic, dilated, restrictive, and arrhythmogenic cardiomyopathies. Translation of this research to the clinic via genetic testing can precisely group affected patients according to molecular etiology, and identify individuals without evidence of disease who are at high risk for developing cardiomyopathy...
December 27, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27999550/the-use-of-berlin-heart-excor-vad-in-children-less-than-10-kg-a-single-center-experience
#19
Arianna Di Molfetta, Fabrizio Gandolfo, Sergio Filippelli, Gianluigi Perri, Luca Di Chiara, Roberta Iacobelli, Rachele Adorisio, Isabella Favia, Alessandra Rizza, Giuseppina Testa, Matteo Di Nardo, Antonio Amodeo
Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27989285/my-approach-to-the-evaluation-of-restrictive-cardiomyopathy
#20
Craig R Asher, Allan L Klein
No abstract text is available yet for this article.
January 2017: Trends in Cardiovascular Medicine
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