Distinguishing Constrictive Pericarditis From Restrictive Cardiomyopathy-An Ongoing Diagnostic Challenge.
Sign Up to like & getrecommendations! Published in 2021 at "JAMA cardiology"
DOI: 10.1001/jamacardio.2021.3483
Abstract: Constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) are serious disorders that share common pathophysiologic elements including biventricular diastolic dysfunction, elevated biatrial pressures, and reduced resting cardiac output. Yet,while these 2 disorders have similar clinical presentations,… read more here.
Keywords: cardiology; pericarditis restrictive; restrictive cardiomyopathy; constrictive pericarditis ... See more keywords
Restrictive cardiomyopathy: an unusual phenotype of a lamin A variant
Sign Up to like & getrecommendations! Published in 2018 at "ESC Heart Failure"
DOI: 10.1002/ehf2.12294
Abstract: 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… read more here.
Keywords: cardiomyopathy unusual; phenotype lamin; unusual phenotype; cardiomyopathy ... See more keywords
Epigenetic regulation of phosphodiesterase 4d in restrictive cardiomyopathy mice with cTnI mutations
Sign Up to like & getrecommendations! Published in 2019 at "Science China Life Sciences"
DOI: 10.1007/s11427-018-9463-9
Abstract: Epigenetic regulations play an important role in disease development. In this study, we have investigated epigenetic regulations in restrictive cardiomyopathy mice with cTnI 193His mutation. Our results demonstrated that phosphodiesterase (PDEs) 4d was down-regulated in… read more here.
Keywords: ctni mutations; cardiomyopathy mice; mice; mice ctni ... See more keywords
Update on restrictive cardiomyopathy
Sign Up to like & getrecommendations! Published in 2017 at "Paediatrics and Child Health"
DOI: 10.1016/j.paed.2017.10.002
Abstract: Abstract Restrictive cardiomyopathy (RCM) is a rare form of heart muscle disease accounting for just 2 to 5% of paediatric cardiomyopathies. It is characterised by increased myocardial stiffness and restrictive filling pattern in to the… read more here.
Keywords: update restrictive; rcm; restrictive cardiomyopathy;
Dysregulated autophagy in restrictive cardiomyopathy due to Pro209Leu mutation in BAG3.
Sign Up to like & getrecommendations! Published in 2018 at "Molecular genetics and metabolism"
DOI: 10.1016/j.ymgme.2018.01.001
Abstract: Myofibrillary myopathies (MFM) are hereditary myopathies histologically characterized by degeneration of myofibrils and aggregation of proteins in striated muscle. Cardiomyopathy is common in MFM but the pathophysiological mechanisms are not well understood. The BAG3-Pro209Leu mutation… read more here.
Keywords: bag3 pro209leu; restrictive cardiomyopathy; pro209leu mutation; mutation ... See more keywords
Molecular Characterization of Pediatric Restrictive Cardiomyopathy from Integrative Genomics
Sign Up to like & getrecommendations! Published in 2017 at "Scientific Reports"
DOI: 10.1038/srep39276
Abstract: 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… read more here.
Keywords: rcm; pediatric restrictive; pediatric rcm; restrictive cardiomyopathy ... See more keywords
Restrictive cardiomyopathy: definition and diagnosis
Sign Up to like & getrecommendations! Published in 2022 at "European Heart Journal"
DOI: 10.1093/eurheartj/ehac543
Abstract: Abstract Restrictive cardiomyopathy (RCM) is a heterogeneous group of diseases characterized by restrictive left ventricular pathophysiology, i.e. a rapid rise in ventricular pressure with only small increases in filling volume due to increased myocardial stiffness.… read more here.
Keywords: restrictive cardiomyopathy; pathophysiology; restrictive pathophysiology; cardiomyopathy definition ... See more keywords
P904Heart rhythm and conduction disorders in children with restrictive cardiomyopathy
Sign Up to like & getrecommendations! Published in 2019 at "European Heart Journal"
DOI: 10.1093/eurheartj/ehz747.0500
Abstract: Restrictive cardiomyopathy (RCMP) is characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal systolic function and wall thickness. It may occur idiopathically or as a cardiac manifestation… read more here.
Keywords: heart; rhythm conduction; conduction disorders; restrictive cardiomyopathy ... See more keywords
Successful treatment of severe combined post- and pre-capillary pulmonary hypertension in a patient with idiopathic restrictive cardiomyopathy
Sign Up to like & getrecommendations! Published in 2018 at "Pulmonary Circulation"
DOI: 10.1177/2045894018770131
Abstract: Restrictive cardiomyopathy (RCM) is a rare form of cardiomyopathy that is characterized by restrictive ventricular filling. Elevated filling pressure leads to pulmonary hypertension (PH), which often progresses to combined post- and pre-capillary PH (Cpc-PH) with… read more here.
Keywords: dpg; heart; pulmonary hypertension; treatment ... See more keywords
Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling.
Sign Up to like & getrecommendations! Published in 2022 at "Reviews in cardiovascular medicine"
DOI: 10.31083/j.rcm2303108
Abstract: Restrictive cardiomyopathy (RCM), a potentially devastating heart muscle disorder, is characterized by diastolic dysfunction due to abnormal muscle relaxation and myocardial stiffness resulting in restrictive filling of the ventricles. Diastolic dysfunction is often accompanied by… read more here.
Keywords: genetics clinical; restrictive cardiomyopathy; clinical overview; cardiomyopathy ... See more keywords
Hgs Deficiency Caused Restrictive Cardiomyopathy via Disrupting Proteostasis
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Biological Sciences"
DOI: 10.7150/ijbs.69024
Abstract: The molecular mechanisms underlying restrictive cardiomyopathy (RCM) are not fully understood. Hepatocyte growth factor-regulated tyrosine kinase substrate (HGS) is a vital element of Endosomal sorting required for transport (ESCRT), which mediates protein sorting for degradation… read more here.
Keywords: hgs; restrictive cardiomyopathy; hgs deficiency; rcm ... See more keywords