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Restrictive Cardiomyopathy

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2. Restrictive cardiomyopathy: an unusual phenotype of a lamin A variant Full Text available with Trip Pro

Restrictive cardiomyopathy: an unusual phenotype of a lamin A variant 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.© 2018 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

2018 ESC heart failure

3. Cardiac and peripheral autonomic control in restrictive cardiomyopathy Full Text available with Trip Pro

Cardiac and peripheral autonomic control in restrictive cardiomyopathy Autonomic dysfunction determines the advance of dilated cardiomyopathy (DCM) and is related to poor outcomes. However, this autonomic imbalance is unknown in patients with restrictive cardiomyopathy (RCM) even though they have similar symptoms and poor quality of life as DCM patients have. The aim of this study was to evaluate if autonomic and neurovascular controls were altered in RCM patients.Fifteen RCM patients, 10 DCM (...) patients, and 10 healthy subjects were evaluated. Heart rate and blood pressure (BP) were recorded. Peripheral sympathetic activity [muscle sympathetic nerve activity (MSNA)] by microneurography and cardiac sympathetic activity by power spectrum analysis of heart rate variability. Spontaneous baroreflex sensitivity (BRS) was evaluated by the sequence method and forearm blood flow by venous occlusion plethysmography. Both cardiomyopathy groups had higher MSNA frequency (P < 0.001) and MSNA incidence (P

2017 ESC heart failure

4. Allele-Specific Silencing Ameliorates Restrictive Cardiomyopathy Attributable to a Human Myosin Regulatory Light Chain Mutation. Full Text available with Trip Pro

Allele-Specific Silencing Ameliorates Restrictive Cardiomyopathy Attributable to a Human Myosin Regulatory Light Chain Mutation. Restrictive cardiomyopathy is a rare heart disease associated with mutations in sarcomeric genes and with phenotypic overlap with hypertrophic cardiomyopathy. There is no approved therapy directed at the underlying cause. Here, we explore the potential of an interfering RNA (RNAi) therapeutic for a human sarcomeric mutation in MYL2 causative of restrictive (...) preserved, suggesting that the RNAi pathway was not saturated.Our results show the feasibility, efficacy, and safety of RNAi therapeutics directed towards human restrictive cardiomyopathy. This is a promising step toward targeted therapy for a prevalent human disease.

2019 Circulation

5. A case report of recessive restrictive cardiomyopathy caused by a novel mutation in cardiac troponin I (TNNI3). Full Text available with Trip Pro

A case report of recessive restrictive cardiomyopathy caused by a novel mutation in cardiac troponin I (TNNI3). Restrictive cardiomyopathy is a rare cardiac disease, for which several genes including TNNT2, MYPN, FLNC and TNNI3 have been associated with its familial form.Here we describe a female proband with a severely manifested restrictive phenotype leading to heart transplantation at the age of 41, who was found homozygous for the novel TNNI3 mutation: NM_000363.4:c.586G > C, p.(Asp196His (...) ). Her parents were third-degree cousins originating from a small village and although they were found heterozygous for the same variant they displayed no symptoms of the disease. Her older sister who was also found heterozygous was asymptomatic. Her twin sister and her brother who were homozygous for the same variant displayed a restrictive and a hypertrophic phenotype, respectively. Their children are all carriers of the mutation and remain asymptomatic until the age of 21.These observations point

2019 BMC Medical Genetics

6. Correction to: Regulation of diabetic cardiomyopathy by caloric restriction is mediated by intracellular signaling pathways involving ‘SIRT1 and PGC-1α’ Full Text available with Trip Pro

Correction to: Regulation of diabetic cardiomyopathy by caloric restriction is mediated by intracellular signaling pathways involving ‘SIRT1 and PGC-1α’ Unfortunately, after publication of this article [1], it was noticed that Table 1 contained errors introduced during the production process. In the WT + AT column, the FS value is 21 ± 7 and the Body Weight value is 25 ± 2. In the WT + AT + CR column, the FS value is 46 ± 14 and the Body Weight value is 19 ± 1. The original article has

2018 Cardiovascular diabetology

7. Regulation of diabetic cardiomyopathy by caloric restriction is mediated by intracellular signaling pathways involving ‘SIRT1 and PGC-1α’ Full Text available with Trip Pro

Regulation of diabetic cardiomyopathy by caloric restriction is mediated by intracellular signaling pathways involving ‘SIRT1 and PGC-1α’ Metabolic disorders such as obesity, insulin resistance and type 2 diabetes mellitus (DM2) are all linked to diabetic cardiomyopathy that lead to heart failure. Cardiomyopathy is initially characterized by cardiomyocyte hypertrophy, followed by mitochondrial dysfunction and fibrosis, both of which are aggravated by angiotensin. Caloric restriction (CR (...) ) is cardioprotective in animal models of heart disease through its catabolic activity and activation of the expression of adaptive genes. We hypothesized that in the diabetic heart; this effect involves antioxidant defenses and is mediated by SIRT1 and the transcriptional coactivator PGC-1α (Peroxisome proliferator-activated receptor-γ coactivator).Obese Leptin resistant (db/db) mice characterized by DM2 were treated with angiotensin II (AT) for 4 weeks to enhance the development of cardiomyopathy. Mice were

2018 Cardiovascular diabetology

8. Impact of Exercise Restriction on Arrhythmic Risk Among Patients With Arrhythmogenic Right Ventricular Cardiomyopathy Full Text available with Trip Pro

Impact of Exercise Restriction on Arrhythmic Risk Among Patients With Arrhythmogenic Right Ventricular Cardiomyopathy Prior studies have shown a close link between exercise and development of arrhythmogenic right ventricular cardiomyopathy. How much exercise restriction reduces ventricular arrhythmia (VA), how genotype modifies its benefit, and whether it reduces risk sufficiently to defer implantable cardioverter-defibrillator (ICD) placement in arrhythmogenic right ventricular cardiomyopathy (...) =0.16 and P=0.06 for interaction); however, 58% (18/31) of athletes who reduced exercise dose by >80% still experienced VA.Exercise restriction should be recommended to all arrhythmogenic right ventricular cardiomyopathy patients with ICDs. Patients who are "gene-elusive" and those with primary-prevention devices may particularly benefit. Exercise reduction is unlikely to reduce arrhythmia sufficiently in high-risk patients to alter decision-making regarding ICD implantation.© 2018 The Authors

2018 Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

9. Cardiomyocyte-Restricted Low Density Lipoprotein Receptor-Related Protein 6 (LRP6) Deletion Leads to Lethal Dilated Cardiomyopathy Partly Through Drp1 Signaling Full Text available with Trip Pro

Cardiomyocyte-Restricted Low Density Lipoprotein Receptor-Related Protein 6 (LRP6) Deletion Leads to Lethal Dilated Cardiomyopathy Partly Through Drp1 Signaling Low density lipoprotein receptor-related protein 6 (LRP6), a wnt co-receptor, regulates multiple functions in various organs. However, the roles of LRP6 in the adult heart are not well understood. Methods: We observed LRP6 expression in heart with end-stage dilated cardiomyopathy (DCM) by western blot. Tamoxifen-inducible cardiac (...) -specific LRP6 knockout mouse was constructed. Hemodynamic and echocardiographic analyses were performed to these mice. Results: Cardiac LRP6 expression was dramatically decreased in patients with end-stage dilated cardiomyopathy (DCM) compared to control group. Tamoxifen-inducible cardiac-specific LRP6 knockout mice developed acute heart failure and mitochondrial dysfunction with reduced survival. Proteomic analysis suggests the fatty acid metabolism disorder involving peroxisome proliferator-activated

2018 Theranostics

10. Successful treatment of severe combined post- and pre-capillary pulmonary hypertension in a patient with idiopathic restrictive cardiomyopathy Full Text available with Trip Pro

Successful treatment of severe combined post- and pre-capillary pulmonary hypertension in a patient with idiopathic restrictive cardiomyopathy 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 increased diastolic pulmonary vascular pressure gradient (DPG) and pulmonary vascular

2018 Pulmonary circulation

11. Mechanical Circulatory Support Device Utilization and Heart Transplant Waitlist Outcomes in Patients With Restrictive and Hypertrophic Cardiomyopathy. Full Text available with Trip Pro

Mechanical Circulatory Support Device Utilization and Heart Transplant Waitlist Outcomes in Patients With Restrictive and Hypertrophic Cardiomyopathy. 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

2018 Circulation. Heart failure

12. Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series. Full Text available with Trip Pro

Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series. Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive (...) cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, particularly primary and secondary forms of iron overload, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

2018 Journal of the American College of Cardiology

13. Idiopathic Restrictive Cardiomyopathy in Children and Young Adults. (Abstract)

Idiopathic Restrictive Cardiomyopathy in Children and Young Adults. Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975

2018 American Journal of Cardiology

14. Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 1 of a 2-Part Series. Full Text available with Trip Pro

Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 1 of a 2-Part Series. Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive (...) cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, including lysosomal and glycogen storage disorders, iron overload, and amyloidosis (both light chain amyloidosis and transthyretin amyloidosis variants), as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.Copyright © 2018

2018 Journal of the American College of Cardiology

15. BAG3-related myofibrillar myopathy requiring heart transplantation for restrictive cardiomyopathy Full Text available with Trip Pro

BAG3-related myofibrillar myopathy requiring heart transplantation for restrictive cardiomyopathy 30023292 2018 11 14 2214-4269 15 2018 Jun Molecular genetics and metabolism reports Mol Genet Metab Rep BAG3 -related myofibrillar myopathy requiring heart transplantation for restrictive cardiomyopathy. 65-66 10.1016/j.ymgmr.2018.02.002 Finsterer Josef J Krankenanstalt Rudolfstiftung, Vienna, Austria. Zarrouk-Mahjoub Sinda S University of Tunis El Manar, Genomics Platform, Pasteur Institute

2018 Molecular genetics and metabolism reports

16. Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience. (Abstract)

Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience. Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx.All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy (...) with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Outcomes of those listed for HTx (N = 18) were compared to a benchmark of contemporaneous pediatric RCM patients in the UNOS database (N = 377). Proportional hazards models were used to determine predictors of adverse outcomes.The mean age was 11 ± 9 years and 49% were male. 14 of 18 patients listed received HTx. Overall mortality (12%) was identical between the phenotypes; however, RCM patients were

2018 Pediatric Cardiology

17. Assessment of cardiomyopathy

is predominantly confined to the heart muscle and where subclassifications of genetic, mixed, and acquired are adopted. Secondary cardiomyopathies are those where myocardial involvement occurs as part of a systemic or multi-organ disorder. It is important to recognise that the traditional classification into hypertrophic, dilated, and restrictive cardiomyopathies mixes anatomical with functional designations, which are not mutually exclusive. [Figure caption and citation for the preceding image starts (...) ]: Proposed classification system Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008;29:270-276. Used with permission. [Citation ends]. Differentials Idiopathic dilated cardiomyopathy Myocarditis Alcohol: dilated cardiomyopathy Hypertrophic cardiomyopathy (HCM) Idiopathic restrictive cardiomyopathy Arrhythmogenic right ventricular

2018 BMJ Best Practice

18. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy Full Text available with Trip Pro

, sudden death risk, prognosis, and focus of management are different in these two scenarios. Although rare, ACM can also overlap with hypertrophic cardiomyopathy (HCM; final common pathway, the sarcomere), restrictive cardiomyopathy (RCM; final common pathway, the sarcomere), or LV noncompaction (LVNC; final common pathway, the sarcomere and cytoskeleton). Troponin T variants, unlike other sarcomeric disease-causing genes, may present with cardiac arrest or sudden death despite mild or even absent LV (...) hypertrophy, whereas troponin I variants may cause a restrictive phenotype in which the dominant clinical presentation is atrial fibrillation. x 13 McKenna, W.J., Stewart, J.T., Nihoyannopoulos, P., McGinty, F., and Davies, M.J. Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass. Br Heart J . 1990 ; 63 : 287–290 | | | , x 14 Watkins, H., Ashrafian, H., and Redwood, C. Inherited cardiomyopathies. N Engl J Med . 2011 ; 364

2019 International Society for Heart and Lung Transplantation

19. Genetic Evaluation of cardiomyopathy

available after that date. It also would be prudent to consider whether intellectual property interests may restrict the performance of certain tests and other procedures. Purpose: The purpose of this document is to provide updated guidance for the genetic evaluation of cardiomyopathy and for an approach to manage secondary findings from cardiomyopathy genes. The genetic bases of the primary cardiomyopathies (dilated, hypertrophic, arrhythmogenic right ventricular, and restrictive) have been established (...) (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ven- tricular cardiomyopathy (ARVC), and restrictive cardiomyo- pathy (RCM). Despite this complex interplay of genes, variants, and phenotypes, current knowledge when combined with expert phenotyping and the sensitivity and specificity of current genetic testing is sufficient to effectively conduct genetic cardiomyopathy evaluations. We caution, however, that variant interpretation must be thoughtful, rigorous, and leverage the most up

2019 American College of Medical Genetics and Genomics

20. Magnetic resonance imaging of patients with suspected non-ischaemic cardiomyopathies

increased left ventricular [LV]) wall thickness, a hypertrophic or restrictive cardiomyopathy [HCM or RCM] is suspected, and further diagnostic clarification is required) and population 2 (asymptomatic individuals with a first-degree relative with HCM/RCM with increased or indeterminate left ventricular wall thickness on echocardiography). MSAC noted that evidence to support the use of CMR in these populations may have been missed and suggested a new application to identify additional evidence (...) covered hypertrophic cardiomyopathies (HCM), restrictive cardiomyopathies (RCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and cardiomyopathies that may be due to acute coronary syndrome (ACS), myocarditis or Takotsubo cardiomyopathy (TTC). MSAC noted that Part A which was the application for patients with dilated cardiomyopathies (DCM) was considered at the July 2016 meeting (MSAC Public Summary Document (PSD) Application 1393, July 2016). MSAC noted that cardiomyopathies are diseases

2017 Medical Services Advisory Committee

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