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endomyocardial fibrosis

Takeo Fujino, Shujiro Inoue, Shunsuke Katsuki, Taiki Higo, Tomomi Ide, Yoshinao Oda, Hiroyuki Tsutsui
A 31-year-old man was admitted to our hospital with atrial tachycardia and cardiogenic shock. He had been diagnosed with hereditary spherocytosis (HS) during childhood, but he never received any red blood cell transfusions. Right ventricular endomyocardial biopsy revealed multiple myocardial hemosiderin deposits, and he was diagnosed with cardiac hemochromatosis. In addition to the iron deposition in the heart, the loss of myocyte and severe interstitial fibrosis were present. His cardiac function did not improve even after the cardioversion for atrial tachycardia, and he suffered from recurrent heart failure...
March 20, 2018: International Heart Journal
Ming Gao, Weihua Zhang, Waiou Zhao, Ling Qin, Fei Pei, Yang Zheng
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis." PATIENT CONCERNS: We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy...
March 2018: Medicine (Baltimore)
Petr Kuchynka, Tomas Palecek, Lukas Lambert, Martin Masek, Vaclav Ptacnik
No abstract text is available yet for this article.
February 23, 2018: European Heart Journal Cardiovascular Imaging
Paul R Scully, Gorka Bastarrika, James C Moon, Thomas A Treibel
PURPOSE OF REVIEW: This review article discusses the evolution of extracellular volume (ECV) quantification using both cardiovascular magnetic resonance (CMR) and computed tomography (CT). RECENT FINDINGS: Visualizing diffuse myocardial fibrosis is challenging and until recently, was restricted to the domain of the pathologist. CMR and CT both use extravascular, extracellular contrast agents, permitting ECV measurement. The evidence base around ECV quantification by CMR is growing rapidly and just starting in CT...
March 6, 2018: Current Cardiology Reports
Prabha Nini Gupta, Subair M Kunju, Baiju Rajan, A George Koshy, Sunitha Vishwanathan, Preeti Sara George, Praveen Velappan
OBJECTIVES: To compare the clinical presentation, clinical profile and survival of two groups of endomyocardial fibrosis patients. METHODS: The study was a prospective cohort study, or a prospective case series, comparing all consecutive echocardiographically proven patients with endomyocardial fibrosis seen in Medical College Trivandrum with the patients seen in Medical College Hospital, Alappuzha(Alleppey) (or TD Medical College). In all patients the clinical details like age, sex, type of endomyocardial fibrosis, the presence of anaemia, eosinophilia, neutrophilia and type of rhythm(Sinus or atrial fibrillation) etc were compared by both simple X2 and by Kaplan Meier survival curves...
January 2018: Indian Heart Journal
Job A J Verdonschot, Mark R Hazebroek, Kasper W J Derks, Arantxa Barandiarán Aizpurua, Jort J Merken, Ping Wang, Jörgen Bierau, Arthur van den Wijngaard, Simon M Schalla, Myrurgia A Abdul Hamid, Marc van Bilsen, Vanessa P M van Empel, Christian Knackstedt, Hans-Peter Brunner-La Rocca, Han G Brunner, Ingrid P C Krapels, Stephane R B Heymans
Aims: Truncating titin variants (TTNtv) are the most prevalent genetic cause of dilated cardiomyopathy (DCM). We aim to study clinical parameters and long-term outcomes related to the TTNtv genotype and determine the related molecular changes at tissue level in TTNtv DCM patients. Methods and results: A total of 303 consecutive and extensively phenotyped DCM patients (including cardiac imaging, Holter monitoring, and endomyocardial biopsy) underwent DNA sequencing of 47 cardiomyopathy-associated genes including TTN, yielding 38 TTNtv positive (13%) patients...
January 25, 2018: European Heart Journal
Ana Luiza C Sayegh, Marcelo R Dos Santos, Patricia de Oliveira, Fábio Fernandes, Eduardo Rondon, Francis R de Souza, Vera M C Salemi, Maria Janieire de N N Alves, Charles Mady
BACKGROUND: Endomyocardial fibrosis (EMF) is a rare disease, characterized by diastolic dysfunction which leads to reduced peak oxygen consumption (VO2). Cardiopulmonary exercise testing (CPET) has been proved to be a fundamental tool to identify central and peripheral alterations. However, most studies prioritize peak VO2 as the main variable, leaving aside other important CPET variables that can specify the severity of the disease and guide the clinical treatment. OBJECTIVE: The aim of this study was to evaluate central and peripheral limitations in symptomatic patients with EMF by different CPET variables...
December 2017: Arquivos Brasileiros de Cardiologia
Steven Hsu, Kristen M Kokkonen-Simon, Jonathan A Kirk, Todd M Kolb, Rachel L Damico, Stephen C Mathai, Monica Mukherjee, Ami A Shah, Fredrick M Wigley, Kenneth B Margulies, Paul M Hassoun, Marc K Halushka, Ryan J Tedford, David A Kass
Background -Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility as compared to IPAH. We tested whether this disparity involves underlying differences in myofilament function. Methods -Cardiac myocytes were isolated from RV septal endomyocardial biopsies from patients with SSc-PAH, IPAH, or SSc with exertional dyspnea but no resting PAH (SSc-d); control RV septal tissue was obtained from non-diseased donor hearts (6-7/group)...
January 19, 2018: Circulation
Parul Jain, Sudheer Arava, Sandeep Seth, Sanjeev Lalwani, Ruma Ray
INTRODUCTION: Collagen distribution alterations are well known in dilated cardiomyopathy. There are also changes in microvasculature along with other histomorphorphological features. AIMS AND OBJECTIVES: To study the histomorphological features of DCM along with their quantitative correlation with LVEF. Alterations in collagen IV distribution pattern and microvasculature in DCM were also evaluated. MATERIALS AND METHODS: The present study includes 34 right ventricular endomyocardial biopsies, 7 explanted native hearts and 41 autopsy control hearts...
October 2017: Indian Journal of Pathology & Microbiology
Hannes Lindemann, Ivana Petrovic, Stephan Hill, Anastasios Athanasiadis, Heiko Mahrholdt, Tim Schäufele, Karin Klingel, Udo Sechtem, Peter Ong
BACKGROUND: Patients with angina yet having unobstructed coronaries are found in ∼50% of cases undergoing invasive angiography. Coronary spasm and microvascular dysfunction can be responsible for the clinical presentation in ∼60% of cases. However, little is known about structural changes in the myocardium. The aim of this study was to describe findings in endomyocardial biopsies of symptomatic patients with unobstructed coronaries. PATIENTS AND METHODS: We retrospectively analyzed a cohort of 1416 consecutive patients who underwent endomyocardial biopsy sampling and coronary angiography between 2002 and 2016 for various clinical indications...
January 8, 2018: Coronary Artery Disease
Nicholas Child, Gonca Suna, Darius Dabir, May-Lin Yap, Toby Rogers, Misha Kathirgamanathan, Eduardo Arroyo-Ucar, Rocio Hinojar, Islam Mahmoud, Christopher Young, Olaf Wendler, Manuel Mayr, Banher Sandhu, Geraint Morton, Marion Muhly-Reinholz, Stefanie Dimmeler, Eike Nagel, Valentina O Puntmann
Aims: To determine the bioequivalence of several T1 mapping sequences in myocardial characterization of diffuse myocardial fibrosis. Methods and results: We performed an intra-individual sequence comparison of three types of T1 mapping sequences [MOdified Look-Locker Inversion recovery (MOLLI), Shortened MOdified Look-Locker Inversion recovery ((sh)MOLLI), and SAturation recovery single-SHot Acquisition (SASHA)]. We employed two model diseases of diffuse interstitial fibrosis [patients with non-ischaemic dilated cardiomyopathy (NIDCM), n = 32] and aortic stenosis [(AS), n = 25)]...
December 11, 2017: European Heart Journal Cardiovascular Imaging
Suneesh Kalliath, Gopalan Nair Rajesh
A 66-year-old man presented with abdominal distension and pedal oedema for the past 10 years. He had history of right heart failure on several occasions in the past, and one of these admissions prompted referral to a cardiac specialist. On examination, he had markedly elevated jugular venous pressure with prominent 'y-descent', a tricuspid regurgitation murmur, gross ascites and pedal oedema. A full blood count, routine biochemical screen and serum bicarbonate levels were normal. Right ventricular angiogram revealed a 'caterpillar'-like aneurysm of the right ventricle (RV) apex (figure 1 arrow) with a dilated right ventricular outflow tract and significant tricuspid regurgitation with a dilated right atrium (see online supplementary video 1)...
2017: Heart Asia
Christopher C Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau, Luke Y C Chen
Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential...
November 2017: American Journal of the Medical Sciences
Timothy G Norwood, Brian C Westbrook, Douglas B Johnson, Silvio H Litovsky, Nina L Terry, Svetlana B McKee, Alan S Gertler, Javid J Moslehi, Robert M Conry
BACKGROUND: Severe myocarditis associated with electrical conduction abnormalities and occasionally heart failure has been well documented following treatment with immune checkpoint blockade with an estimated incidence of less than 1%. However, the incidence, early detection, and management of less severe immune-related myocarditis are unknown since most immunotherapy trials have not included routine cardiac monitoring. Herein, we provide the first description of subclinical or smoldering myocarditis with minimal signs and symptoms following immune checkpoint blockade with a single dose of ipilimumab and nivolumab...
November 21, 2017: Journal for Immunotherapy of Cancer
Paweł Rubiś, Sylwia Wiśniowska-Śmiałek, Ewa Dziewięcka, Lucyna Rudnicka-Sosin, Artur Kozanecki, Piotr Podolec
INTRODUCTION: Serum markers of fibrosis provide an insight into extracellular matrix (ECM) fibrosis in heart failure (HF) and dilated cardiomyopathy (DCM). However, their role as predictors of cardiovascular (CV) events in DCM is poorly understood. METHODS: This is an observational, prospective cohort study. 70 DCM patients (48±12.1years, ejection fraction - EF 24.4±7.4) were recruited. Markers of collagen type I and III synthesis - procollagen type I and III carboxy- and amino-terminal peptides (PICP, PIIICP, PINP, PIIINP), fibrosis controlling factors - ostepontin (OPN), transforming growth factor (TGF1-β) and connective tissue growth factor (CTGF), and matrix metalloproteinases (MMP-2, MMP-9) and tissue inhibitor (TIMP-1), were measured in serum...
November 6, 2017: Advances in Medical Sciences
Hiroaki Hiraiwa, Takahiro Okumura, Akinori Sawamura, Yuki Sugiura, Toru Kondo, Naoki Watanabe, Soichiro Aoki, Takeo Ichii, Katsuhide Kitagawa, Naoaki Kano, Kenji Fukaya, Kenji Furusawa, Ryota Morimoto, Kyosuke Takeshita, Yasuko K Bando, Toyoaki Murohara
BACKGROUND: Myocardial fibrosis is associated with poor prognosis in nonischemic dilated cardiomyopathy (NIDCM) patients. The Selvester QRS score on 12-lead electrocardiogram is associated with both the amount of myocardial scar and poor prognosis in myocardial infarction patients. However, its use in NIDCM patients is limited. We investigated the prognostic value of the QRS score and its association with collagen volume fraction (CVF) in NIDCM patients. METHODS: We enrolled 91 consecutive NIDCM patients (66 men, 53±13 years) without permanent pacemakers or cardiac resynchronization therapy devices...
October 20, 2017: Journal of Cardiology
Brian Feingold, Jennifer Picarsic, Andrew Lesniak, Benjamin A Popp, Michelle A Wood-Trageser, Anthony J Demetris
BACKGROUND: Histopathologic features of late graft dysfunction (LGD) in endomyocardial biopsies (EMBs) after pediatric heart transplantation (HT) have been incompletely described and rarely quantified. We employed automated, morphometric analysis of whole-slide EMB images to objectively quantify fibrosis and microvasculopathy after pediatric HT. METHODS: Nine recipients with clinical LGD were matched with controls on age, listing diagnosis, crossmatch and time since HT...
December 2017: Journal of Heart and Lung Transplantation
Paulo Sampaio Gutierrez, Fernando Peixoto Ferraz de Campos
No abstract text is available yet for this article.
July 2017: Autopsy & Case Reports
Alexandre Leger, Alexia Hennig, Jerome Peyrou, Hubert Cochet
No abstract text is available yet for this article.
November 1, 2017: European Heart Journal Cardiovascular Imaging
Paula Rozenfeld, Sandro Feriozzi
Lysosomal storage diseases are usually considered to be pathologies in which the passive deposition of unwanted materials leads to functional changes in lysosomes. Lysosomal deposition of unmetabolized glycolipid substrates stimulates the activation of pathogenic cascades, including immunological processes, and particularly the activation of inflammation. In lysosomal storage diseases, the inflammatory response is continuously being activated because the stimulus cannot be eliminated. Consequently, inflammation becomes a chronic process...
November 2017: Molecular Genetics and Metabolism
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