Restrictive Cardiomyopathy Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Multimodality imaging of eosinophilic myocarditis (loeffler ’s endocarditis) in a patient with idiopathic hypereosinophilic syndrome
AbstractEosinophilic myocarditis can result in endomyocardial fibrosis affecting both ventricles, leading to restrictive cardiomyopathy. Multimodality imaging is crucial for diagnosis, as demonstrated in this case of a patient presenting with symptoms of heart failure. (Source: The International Journal of Cardiovascular Imaging)
Source: The International Journal of Cardiovascular Imaging - April 18, 2024 Category: Radiology Source Type: research

Pathophysiology of Cardiac Amyloidosis
Amyloidosis refers to a heterogeneous group of disorders sharing common pathophysiological mechanisms characterized by the extracellular accumulation of fibrillar deposits consisting of the aggregation of misfolded proteins. Cardiac amyloidosis (CA), usually caused by deposition of misfolded transthyretin or immunoglobulin light chains, is an increasingly recognized cause of heart failure burdened by a poor prognosis. CA manifests with a restrictive cardiomyopathy which progressively leads to biventricular thickening, diastolic and then systolic dysfunction, arrhythmias, and valvular disease. The pathophysiology of CA is m...
Source: Heart Failure Clinics - April 8, 2024 Category: Cardiology Authors: Paolo Morfino, Alberto Aimo, Maria Franzini, Giuseppe Vergaro, Vincenzo Castiglione, Giorgia Panichella, Giuseppe Limongelli, Michele Emdin Source Type: research

Preclinical evaluation of Tc-99m p5+14 peptide for SPECT detection of cardiac amyloidosis
Conclusion99mTc-p5+14 was prepared in minutes in>20 mCi doses with good performance in preclinical studies making it suitable for clinical SPECT/CT imaging of cardiac amyloidosis. (Source: PLoS One)
Source: PLoS One - April 5, 2024 Category: Biomedical Science Authors: Stephen J. Kennel Source Type: research

How to use MRI in cardiac disease with diastolic dysfunction?
Br J Radiol. 2024 Apr 4:tqae071. doi: 10.1093/bjr/tqae071. Online ahead of print.ABSTRACTLeft ventricular (LV) diastolic dysfunction (DD) is an initially asymptomatic condition that can progress to heart failure, either with preserved or reduced ejection fraction. As such, DD is a growing public health problem. Impaired relaxation, the first stage of DD, is associated with altered LV filling. With progression, reducing LV compliance leads to restrictive cardiomyopathy. While cardiac magnetic resonance (CMR) imaging is the reference for LV systolic function assessment, transthoracic echocardiography (TTE) with Doppler flow ...
Source: The British Journal of Radiology - April 4, 2024 Category: Radiology Authors: Farah Cadour Adrien Cour Jules Senlis Stanislas Rapacchi Hajer Chennoufi Paul Michelin Colin McQuade Matthieu Demeyere Jean-Nicolas Dacher Source Type: research

One of a Kind: A Rare Case of Cardiac Transplantation in a Patient with Eosinophilic Endomyocardial Fibrosis
Introduction: Eosinophilic myocarditis is an inflammatory cardiomyopathy characterized by eosinophilic infiltration of the myocardium leading to fibrosis. It can be seen in malignancy and immune-mediated disorders. Patients can present acutely with myocarditis or chronically as restrictive cardiomyopathy (RC), known as Loeffler ’s endocarditis (eosinophilic endomyocardial fibrosis). The symptoms may be non-specific, and a definitive diagnosis is made with cardiac Magnetic Resonance Imaging (cMRI) or endomyocardial biopsy. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , K. Girgis, J. Celenza-Salvatore, C. Gidea, F. Ali, M. Montgomery, N. Hochbaum, B. Fyfe-Kirschner, S. Kapoor Source Type: research

Bi-Atrial Inflow Cannulation with BVAD: A Novel Approach for Bridging Restrictive Cardiomyopathy Patients to Heart Transplantation
Introduction: Restrictive cardiomyopathy (RCM) poses a significant challenge in utilizing mechanical circulatory devices (MCD) as a bridge to heart transplantation (HT). Bi-atrial inflow cannulation can be feasible approach for MCD implantation. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , A. Hussain, A. Al Ghamdi, A. Arifi, A. Hussanat, N. Selimovic Source Type: research

Exception Requests and Approval Rates After Implementation of the National Heart Review Board for Pediatrics
Purpose: The OPTN Guidance for pediatric heart transplant exception requests addresses dilated cardiomyopathy (DCM), hypertrophic or restrictive cardiomyopathy (HCM/RCM), single ventricle congenital heart disease (1V CHD), and cardiac allograft vasculopathy (CAV). The purpose of our study was to evaluate the use and approval rates of exception requests after the implementation of the National Heart Review Board (NHRB) for Pediatrics. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , D. Bearl, L. Wright Source Type: research

Ferroptosis in Cardiovascular Disease and Cardiomyopathies: Therapeutic Implications of Glutathione and Iron Chelating Agents
Biomedicines. 2024 Mar 1;12(3):558. doi: 10.3390/biomedicines12030558.ABSTRACTThis review explores ferroptosis, a form of regulated cell death reliant on iron-induced phospholipid peroxidation, in diverse physiological and pathological contexts, including neurodegenerative disorders, and ischemia-reperfusion. In the realm of cardiovascular diseases, it significantly contributes to cardiomyopathies, including dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. Ferroptosis involves intricate interactions within cellular iron metabolism, lipid peroxidation, and the balance between polyunsatura...
Source: Atherosclerosis - March 28, 2024 Category: Cardiology Authors: John Dawi Scarlet Affa Edgar Gonzalez Yura Misakyan David Nikoghosyan Karim Hajjar Samuel Kades Sabrina Fardeheb Hayk Mirzoyan Vishwanath Venketaraman Source Type: research

Neuromuscular and cardiovascular phenotypes in paediatric titinopathies: a multisite retrospective study
Conclusion Our cohort demonstrates the genotype–phenotype spectrum of paediatric-onset titinopathies identified in clinical practice and highlights the risk of life-threatening cardiovascular complications. We show the difficulties of obtaining a molecular diagnosis, particularly in neuromuscular patients, and bring awareness to the complexities of genetic counselling in this population. (Source: Journal of Medical Genetics)
Source: Journal of Medical Genetics - March 21, 2024 Category: Genetics & Stem Cells Authors: Meyer, A. P., Barnett, C. L., Myers, K., Siskind, C. E., Moscarello, T., Logan, R., Roggenbuck, J., Rich, K. A. Tags: Genotype-phenotype correlations Source Type: research

Incidental Finding and Treatment of An Anomalous Aortic Origin of the Right Coronary Artery in a Pediatric Donor ’s Heart prior to Implantation
Coronary artery anomalies in donor hearts are an infrequent incidental finding during intraoperative coronary artery inspection. Herein, we report a pediatric case of restrictive cardiomyopathy with secondary pulmonary hypertension, who underwent a heart transplant, and the procured heart was discovered to have a right coronary artery (RCA) anomaly with anomalous origin from the ascending aorta and with an intramural course. This necessitated RCA unroofing during the transplant. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - March 18, 2024 Category: Transplant Surgery Authors: Fumiya Yoneyama, Iki Adachi, William J Dreyer, Silvana Molossi, Jeffery S. Heinle, Ziyad Binsalamah Tags: Case Anecdotes, Comments and Opinions Source Type: research

Generation of human induced pluripotent stem cell line from a patient with restrictive cardiomyopathy
Stem Cell Res. 2024 Feb 28;76:103370. doi: 10.1016/j.scr.2024.103370. Online ahead of print.ABSTRACTRestrictive cardiomyopathy (RCM) is a rare cardiomyopathy characterized by diastolic dysfunction, which affects cardiac systolic function. We successfully established human induced pluripotent stem cells (hiPSCs) from peripheral blood mononuclear cells of 24-year-old male with restrictive cardiomyopathy (RCM). The patient-derived hiPSCs carried heterozygous mutation of CRYAB gene (c.326A > G, p.D109G), which was consistent with clinical whole exon sequencing results. We confirmed the pluripotency, multipotential different...
Source: Cell Research - March 1, 2024 Category: Cytology Authors: Jingxian Li Jinxiu Jiang Lingqun Ye Zhipeng Lian Hui Gong Wei Lei Yuxiang Dai Shijun Hu Source Type: research

Tropomyosin-troponin complex in inherited cardiomyopathies
The tropomyosin-troponin complex (TTC) regulates cardiac contractility by controlling cross-bridge attachments to actin filaments based on intracellular Ca2+ levels. TTC consists of tropomyosin (TPM), cardiac troponin-T (cTnT), cardiac troponin-I (cTnI), and cardiac troponin-C (cTnC). Pathogenic variants (mutations) in genes encoding these proteins (TPM1 [TPM], TNNT2 [cTnT], TNNI3 [cTnI], and TNNC1 [cTnC]) cause different cardiomyopathy types, including hypertrophic (HCM), dilated (DCM), or restrictive cardiomyopathy (RCM). (Source: Heart Rhythm)
Source: Heart Rhythm - February 19, 2024 Category: Cardiology Authors: Christian Krijger Ju árez, Vasco Sequeira, Malou van den Boogaard, Christiaan C. Veerman, Nicola J. Hoetjes, Edwin Poel, Michael W.T. Tanck, Ronald H. Lekanne Deprez, Alexa M.C. Vermeer, Jolanda van der Velden, Ahmad S. Amin Source Type: research

Tropomyosin –troponin complex in inherited cardiomyopathies
The tropomyosin –troponin complex (TTC) regulates cardiac contractility by controlling cross-bridge attachments to actin filaments based on intracellular Ca2+ levels. TTC consists of tropomyosin (TPM), cardiac troponin-T (cTnT), cardiac troponin-I (cTnI), and cardiac troponin-C (cTnC). Pathogenic variants (mutati ons) in genes encoding these proteins (TPM1 [TPM], TNNT2 [cTnT], TNNI3 [cTnI], and TNNC1 [cTnC]) cause different cardiomyopathy types, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM). (Source: Heart Rhythm)
Source: Heart Rhythm - February 19, 2024 Category: Cardiology Authors: Christian Krijger Ju árez, Vasco Sequeira, Malou van den Boogaard, Christiaan C. Veerman, Nicola J. Hoetjes, Edwin Poel, Michael W.T. Tanck, Ronald H. Lekanne Deprez, Alexa M.C. Vermeer, Jolanda van der Velden, Ahmad S. Amin Tags: Research Letter Source Type: research