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'Endomyocardial Fibrosis' (2683)


Jan
2018

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.
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.

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Dec
2017

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.

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Jan
2018

-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.

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Jan
2018

Collagen distribution alterations are well known in dilated cardiomyopathy. There are also changes in microvasculature along with other histomorphorphological features.
To study the histomorphological features of DCM along with their quantitative correlation with LVEF.

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Jan
2018

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.

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Dec
2017

To determine the bioequivalence of several T1 mapping sequences in myocardial characterization of diffuse myocardial fibrosis.
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)].

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Dec
1969

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.

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Nov
2017

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.

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Nov
2017

The alarmins S100A8 and S100A9 are damage-associated molecular patterns, which play a pivotal role in cardiovascular diseases, inflammation, and viral infections. We aimed to investigate their role in Coxsackievirus B3 (CVB3)-induced myocarditis.
S100A8 and S100A9 mRNA expression was 13.

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Nov
2017

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.

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Nov
2017

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.
This is an observational, prospective cohort study.

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Nov
2017

We investigated the effects and underlying mechanisms of genistein on pathological cardiac hypertrophy in vivo and in vitro.
A cardiac hypertrophy model was developed by aortic banding (AB). C57/BL6 mice were randomly assigned to sham + vehicle (CON), sham + genistein (GEN), AB + vehicle (AB), and AB + genistein groups.

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Mar
2018

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.

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Dec
2017

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.
Nine recipients with clinical LGD were matched with controls on age, listing diagnosis, crossmatch and time since HT.

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Dec
2017

Endomyocardial fibrosis.

Autops Case Rep 2017 Jul-Sep;7(3):3-6. Epub 2017 Sep 30.
Paulo Sampaio Gutierrez, Fernando Peixoto Ferraz de Campos

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Oct
2017

We investigated the effect of Ginsenoside-Rb3 (Rb3) on the endothelial-to-mesenchymal transition (EMT) of cardiac microvascular endothelial cells (CMVECs) following coxsackievirus B3 (CVB3) infection.
CMVECs were infected with 100 TCID50 CVB3 (CVB3 group) or treated with Rb3 (Rb3 group); stably cultured CMVECs were used as control. Cells treated with the Pyk2 inhibitor TAE226 and PI3K inhibitor LY294002 were used for additional experiments.

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Nov
2017

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.

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Sep
2017

The cytoplasmatic pattern recognition receptor, NOD2 (nucleotide-binding oligomerization domain 2), belongs to the innate immune system and is among others responsible for the recognition of single-stranded RNA. With Coxsackievirus B3 (CVB3) being a single-stranded RNA virus, and the recent evidence that the NOD2 target, NLRP3 (NOD-like receptor family, pyrin domain containing 3) is of importance in the pathogenesis of CVB3-induced myocarditis, we aimed to unravel the role of NOD2 in CVB3-induced myocarditis.
Endomyocardial biopsy NOD2 mRNA expression was higher in CVB3-positive patients compared with patients with myocarditis but without evidence of persistent CVB3 infection.

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Sep
2017

Inflammatory activation occurs in nearly all forms of myocardial injury. In contrast, inflammatory cardiomyopathies refer to a diverse group of disorders in which inflammation of the heart (or myocarditis) is the proximate cause of myocardial dysfunction, causing injury that can range from a fully recoverable syndrome to one that leads to chronic remodeling and dilated cardiomyopathy. The most common cause of inflammatory cardiomyopathies in developed countries is lymphocytic myocarditis most commonly caused by a viral pathogenesis.

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Sep
2017

Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan.

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Dec
1969

Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention.

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Sep
2017

We describe the case of a 24-year-old male with hyper-IgE syndrome (HIES) which was diagnosed at 4 years of age and died from a very rare cardiac complication. He had typical clinical and laboratory manifestations of HIES, including total serum IgE as high as > 100,000 IU/mL. Stem cell transplantation was not available.

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Aug
2017

The relationship between right ventricle (RV), extracellular matrix (ECM) fibrosis and fibrosis-linked, circulating microRNAs in dilated cardiomyopathy (DCM) is unknown.
To uncover the associations between serum markers of ECM metabolism and circulating microRNAs with RV morphological and functional parameters.
The study population consisted of 70 consecutive DCM patients (ejection fraction 24.

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Dec
1969

The burden of illness associated with acquired cardiac disease in children in low-income and middle-income countries (LMIC) is significant and may be equivalent to that of congenital heart disease. Rheumatic heart disease, endomyocardial fibrosis, cardiomyopathy (including HIV cardiomyopathy) and tuberculosis are the most important causes. All are associated with poverty with the neediest children having the least access to care.

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Dec
1969

Hyperglycemia plays a crucial role in the pathogenesis of diabetic complications; however, the mechanisms underlying diabetic cardiac fibrosis remain unclear. Endothelial cells are known to contribute to cardiac fibrosis through endothelial-mesenchymal transition (EndMT) under high glucose stimulation. Here we investigated the expression of miR-18a-5p and examined its functional role in human aortic valvular endothelial cells (HAVECs).

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Dec
1969

Canine parvovirus-2 (CPV-2) is nearly indistinguishable from feline panleukopenia virus (FPV) and is a well-known cause of viral myocarditis in young puppies; however, it is not known whether either FPV or CPV-2 naturally infects feline cardiomyocytes and causes myocarditis. Endomyocarditis (EMC) and left ventricular endomyocardial fibrosis (LVEF), clinically known as "endomyocardial restrictive cardiomyopathy," are important feline heart diseases suspected to have an infectious etiology. A continuum is suggested with EMC representing the acute reaction to an unknown infectious agent and LVEF the chronic manifestation of repair.

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Jun
2017

Inhibitor of DNA binding (Id) proteins play important roles in regulating cardiac development via paracrine signaling. Id1/Id3 knockout mice die at mid-gestation with multiple cardiac defects. Single Id knockout studies have not reported cardiomyopathies.

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Jun
2017

Eosinophilic myocarditis (EM) is a rare condition that may result from several heterogeneous eosinophilic diseases, including parasite infection, hypersensitivity reaction, vasculitis, and hypereosinophilic syndrome. Regardless of etiology, the disease may present with various cardiac conditions, such as acute coronary syndrome, heart failure, or arrhythmia. Irreversible endomyocardial fibrosis, which causes restrictive cardiomyopathy, occurs in the late phase of the disease.

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Jun
2017

This study aimed to assess characteristics including endomyocardial biopsy and outcome of patients with methamphetamine (MA)-associated cardiomyopathy in a series of patients treated in Germany.
MA abuse is an increasing problem worldwide.
The cases of 30 consecutive MA-abusing patients with a left ventricular (LV) ejection fraction of <40% and endomyocardial biopsy performed at initial diagnosis were analyzed.

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May
2017

Myocardial fibrosis in congenital and pediatric heart disease.

Exp Ther Med 2017 May 10;13(5):1660-1664. Epub 2017 Mar 10.
Jing Tian, Xinjiang An, Ling Niu
Cardiac fibrosis is a common phenomenon in different types of heart diseases, such as ischemic heart disease, inherited cardiomyopathy mutations, diabetes, and ageing and is associated with morbidity and mortality. Increased accumulation of extracellular matrix (ECM) that impacts cardiac function, is the underlying cause of fibrotic heart disease. There are four different types of cardiac fibrosis, including, reactive interstitial fibrosis, replacement fibrosis, infiltrative interstitial fibrosis and endomyocardial fibrosis.

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Jul
2017

Astragaloside Ⅳ (ASG-Ⅳ, (Fig. 1) is the most active component of Chinese sp. Astragalus membranaceus Bunge (Fabaceae) that has showed antioxidant, antiapoptotic and antiviral activities among others.

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Dec
1969

Sickle cell anemia (SCA)-related cardiomyopathy is characterized by diastolic dysfunction and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA. Diastolic dysfunction is associated with microscopic myocardial fibrosis in SCA mice, but the cause of diastolic dysfunction in humans with SCA is unknown.

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Dec
1969

Myocardial fibrosis is an important part of cardiac remodeling that leads to heart failure and death. Myocardial fibrosis results from increased myofibroblast activity and excessive extracellular matrix deposition. Various cells and molecules are involved in this process, providing targets for potential drug therapies.

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Dec
1969

The dynamics of the extracellular matrix (ECM) fibrosis process in dilated cardiomyopathy (DCM) may be assessed non-invasively by means of serum markers of fibrosis.
To explore the kinetics of serum markers of fibrosis during a 12-month follow-up in DCM.
We included 70 consecutive DCM patients (pts) (48±12.

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May
2017

Tachycardiomyopathy or tachycardia-induced cardiomyopathy (TCM) has been known for decades as a reversible form of nonischemic cardiomyopathy. However, its mechanism and properties remain poorly understood.
The current study investigated endomyocardial biopsy samples from patients with TCM and compared them with samples from patients with dilated cardiomyopathy (DCM) and inflammatory cardiomyopathy (ICM).

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Dec
1969

Cardiovascular diseases are widely distributed throughout the world. Human parasitic infections are ubiquitous. Tropical parasites are increasingly recognized as causes of cardiovascular diseases.

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Jan
2018

The aim of the present study was to evaluate whether extracellular volume fraction (ECV) can reliably inform on the extent of diffuse fibrosis in the simultaneous presence of myocardial inflammation, which has not been verified to date.
Diffuse myocardial fibrosis is associated with unfavorable outcome in patients with cardiomyopathy, and is of prognostic relevance. Assessment of ECV bears promise for being a noninvasive surrogate parameter, but it may be altered by other pathologies.

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Jul
2017

To address the mechanisms responsible for the increase in LV filling pressures induced by acute hypertension transients in patients with heart failure with preserved ejection fraction (HFpEF).
Multiple-beat pressure-volume loops were recorded during inferior vena cava occlusion in 39 HFpEF patients and 20 controls during handgrip and atrial pacing. We measured the contribution of relaxation, elastic recoil, and stiffness to instantaneous diastolic pressure using a novel processing method.

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Dec
1969

During acute heart transplant rejection, infiltration of lymphocytes and monocytes is followed by endothelial injury and eventually myocardial fibrosis. To date, no information is available on monocyte-macrophage-related cellular shifts and their polarization status during rejection. Here, we aimed to define and correlate monocyte-macrophage endomyocardial tissue profiles obtained at rejection and time points prior to rejection, with corresponding serial blood samples in 25 heart transplant recipients experiencing acute cellular rejection.

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Apr
2017

The authors previously reported that the c-kit-positive (c-kit) cells isolated from slowly adhering (SA) but not from rapidly adhering (RA) fractions of cardiac mesenchymal cells (CMCs) are effective in preserving left ventricular (LV) function after myocardial infarction (MI).
This study evaluated whether adherence to plastic alone, without c-kit sorting, was sufficient to isolate reparative CMCs.
RA and SA CMCs were isolated from mouse hearts, expanded in vitro, characterized, and evaluated for therapeutic efficacy in mice subjected to MI.

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Dec
1969

We attempted to investigate the therapeutic effects of deferiprone on DC rats and explore the underlying mechanism. Total 24 6-week-old male Wistar rats (weighing from 180 g to 220 g) were subjected to DC model construction and then randomly divided to three groups (8 rats per group): DC group, DC + 50 mg, and DC + 100 mg deferiprone treatment group. The 8 normal rats were considered as controls.

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Mar
2017

Endomyocardial biopsy (EMB) has been established in parallel with the development of percutaneous catheter technology for the diagnosis of cardiac diseases. It was developed in the early 1960 s in Japan by Drs. Konno, Sakakibara and Sekiguchi of Tokyo Women's Medical University.

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Dec
1969

The aim of the study was to characterize the role of Rac1 GTPase for the mineralocorticoid receptor (MR)-mediated pro-fibrotic remodeling. Transgenic mice with cardiac overexpression of constitutively active Rac1 (RacET) develop an age-dependent phenotype with atrial dilatation, fibrosis, and atrial fibrillation. Expression of MR was similar in RacET and WT mice.

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Dec
1969

A 29-year-old man with a history of seizures, was admitted due to an episode of unconsciousness recovered and hypertension with renal disfunction. The electrocardiogram mimicked a hypertrophic cardiomyopathy, but, by Doppler echocardiography, this was discarded because it suggested endomyocardial fibrosis which was confirmed by cardiac magnetic resonance imaging with late enhancement. Since the episode of unconsciousness, brain imaging studies were performed showing vascular sequelae and microangiopathic lesions.

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