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'Cardiomyopathy Hypertrophic' (18263)


Feb
2018

Myocardial fibrosis reside a common pathological feature in hypertrophic and dilated cardiomyopathy that results in ventricular dysfunction leading to heart failure. Though several studies reported the role of fibrosis in cardiac diseases, their pathologic mechanisms leading to heart failure remains unclear. A few studies have proposed integrated analysis of microarray information and protein-protein interaction (PPI) systems to discover subnetwork markers related to diagnosis and prognosis of the disease.

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Feb
2018

Families with a history of hypertrophic cardiomyopathy (HCM) may be offered genetic testing in addition to clinical surveillance. Asymptomatic family members who are gene positive (silent gene carriers) represent a new group of "patients" who may not develop HCM, with little evidence available to assist clinical management. This study explored experiences of HCM genetic testing to identify potential benefits and harms.

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Feb
2018

The prognostic value of high signal intensity on T2-weighted cardiovascular magnetic resonance imaging (T2 high signal) in hypertrophic cardiomyopathy (HCM) patients in a single-center cohort was investigated.Methods and Results:A total of 237 HCM patients (median age, 62 years; 143 male) underwent T2-weighted, cine and late gadolinium enhancement (LGE) imaging, and were followed (median duration, 3.4 years) for life-threatening arrhythmic events.

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Feb
2018

Microvascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy.

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Feb
2018

Left ventricular outflow tract obstruction is observed in 70% of patients with hypertrophic cardiomyopathy, which occurs in about 1 of every 500 adults in the general population. It has been widely believed that the motion of the mitral valve, in particular, its systolic anterior motion (SAM), attributes significantly to such obstruction. For a better understanding of the mitral valve motion, a 3D patient-specific fluid-structure interaction model of the left ventricle from a patient with hypertrophic obstructive cardiomyopathy based on computed tomography (CT) scan images was proposed in this study.

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Dec
2017

The issues of epidemiology and pathophysiology of hypertrophic cardiomyopathy (HCMP), as well as the search for its additional clinical-instrumental and genetic markers, environmental factors capable to influence the formation of its clinical variant and prognosis are subjects of great interest to the modern scientific community. Besides genetic markers of main neurohumoral systems, and morphofunctional parameters of intracardiac hemodynamics clinical course of the disease is influenced by a complex of concomitant pathology including ischemic heart disease (IHD), joining of which is possible in 10% of cases. IHD substantially aggravates course of HCMP and hampers selection of medical therapy.

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Feb
2018

-Compared to transvenous (TV) implantable cardioverter defibrillators (ICD), subcutaneous (S) ICDs require a higher energy for effective defibrillation. Although ventricular fibrillation (VF) conversion testing (CT) is recommended after S-ICD implantation to ensure an adequate margin between the defibrillation threshold and maximum device output (80J), prior work found that adherence to this recommendation is declining.-We studied first time S-ICD recipients (between September 28, 2012 and April 1, 2016) in the National Cardiovascular Database Registry ICD Registry to determine: predictors of use of CT, predictors of an insufficient safety margin (ISM, defined as VF conversion energy >65J) during testing, and in-hospital outcomes associated with use of CT.

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Dec
1969

to investigate the myocardial expression of some structural proteins and markers of cellular proliferation and innate immunity for assessing their possible diagnostic and prognostic role in patients with chronic myocarditis.
The investigation enrolled 23 patients (16 men; mean age, 52.0±12.

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Feb
2018

Children diagnosed with Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (LCHADD) or Very-Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (VLCADD) frequently present with hypertrophic cardiomyopathy or muscle weakness which is caused by the accumulation of fatty acid metabolites due to inactivating mutations in the mitochondrial trifunctional protein. By analyzing mitochondrial morphology we uncovered that mutations within the HADHA or the ACADVL gene not only affect fatty acid oxidation, but also cause significant changes in the DNM1L/MFN2 ratio leading to the significant accumulation of truncated and punctate mitochondria in contrast to network-like mitochondrial morphology in controls. These striking morphological abnormalities correlate with changes in OXPHOS, an imbalance in ROS levels, reduced mitochondrial respiration, reduced growth rates and significantly increased glucose uptake per cell, suggesting that HADHA and ACADVL mutations shift cellular energy household into glycolysis.

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Feb
2018

Segmented phase-sensitive inversion recovery (PSIR) cardiovascular magnetic resonance (CMR) sequences are reference standard for non-invasive evaluation of myocardial fibrosis using late gadolinium enhancement (LGE). Several multi-slice LGE sequences have been introduced for faster acquisition in patients with arrhythmia and insufficient breathhold capability. The aim of this study was to assess the accuracy of several multi-slice LGE sequences to detect and quantify myocardial fibrosis in patients with ischemic and non-ischemic myocardial disease.

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Feb
2018

The relevance of gene mutations leading to heart diseases and hence heart failure has become evident. The risk for and the course of heart failure depends on genomic variants and mutations underlying the so-called genetic predisposition. Genetic contribution to heart failure is highly heterogenous and complex.

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Feb
2018

Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are two diseases with a different pathophysiology, both cause left ventricular hypertrophy (LVH) and myocardial fibrosis. Although remodeling and systolic dysfunction of the left atrium (LA) are associated with atrial fibrillation and stroke in HCM, changes in the size and function of the LA have not been well studied in FD with LVH.
The following groups were studied prospectively, and their respective findings compared: 19 patients with non-obstructive HCM (Group I), 20 patients with a diagnosis of Fabry cardiomyopathy (Group II), and 20 normal subjects matched for sex and age (Group III).

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Dec
1969

Acute bioprosthetic valve thrombosis can occur after surgery and sometimes cause hemodynamic instability and cardiogenic shock. Risk factors for bioprosthetic valve thrombosis are hypercoagulability, atrial fibrillation, atrial dilatation, low cardiac function, and lack of anticoagulation therapy. The authors present a case of severe mitral stenosis due to bioprosthetic valve thrombus.

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Feb
2018

Catecholamines are entrenched in the management of shock states. A paradigm shift has pervaded the critical care arena in recent years acknowledging their propensity to cause harm and fuel a 'death-spiral'. We present the case of a 21-year-old male following a witnessed out-of-hospital cardiac arrest who received high-quality cardiopulmonary resuscitation and standard advanced life support for refractory ventricular fibrillation until return of spontaneous circulation after 70 min.

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Feb
2018

Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young individuals. A potential role of mtDNA mutations in HCM is known. However, the underlying molecular mechanisms linking mtDNA mutations to HCM remain poorly understood due to lack of cell and animal models.

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Feb
2018

Diastolic dysfunction is common in hypertrophic cardiomyopathy (HCM) and hypertensive heart disease (HHD), but its relationships with left ventricular (LV) parameters have not been well studied. Our objective was to assess the relationship of various measures of diastolic function, and maximum left ventricular wall thickness (MLVWT) and left ventricular mass index (LVMI) in HCM, HHD and normal controls using cardiac magnetic resonance imaging (CMR). We also assessed LV parameters and diastolic function in relation to late gadolinium enhancement (LGE) and right ventricular (RV) hypertrophy in HCM.

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Feb
2018

Amyloidosis is caused by deposition of abnormal protein fibrils, leading to damage of organ function. Hereditary amyloidosis represents a monogenic disease caused by germline mutations in 11 amyloidogenic precursor protein genes. One of the important but non-specific symptoms of amyloidosis is hypertrophic cardiomyopathy.

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Feb
2018

Different studies have shown a good long-term survival with improvement of symptoms and hemodynamics after percutaneous septal ablation (PTSMA), similar to myectomy considered to be the therapeutic "gold standard" for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Furthermore, desobliteration of left ventricular (LV) outflow may modify features of the disease considered to be risk-relevant, such as magnitude of LV hypertrophy, outflow gradient, and left atrial (LA) size. The aim oft this study was to examine predictors of long-term mortality in a large cohort of patients with HOCM treated with PTSMA.

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Feb
2018

Intrinsic abnormalities of the mitral valve are common in patients with hypertrophic cardiomyopathy and may need to be addressed at operation.
Consecutive patients undergoing transmitral septal myectomy were retrospectively reviewed. The ventricular septum was exposed through a left atriotomy, and the anterior leaflet of the mitral valve was detached from its annulus.

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Feb
2018

Hypertrophic cardiomyopathy (HCM) is frequently linked to mutations in the protein components of the myosin-containing thick filaments leading to contractile dysfunction and ultimately heart failure. However, the molecular structure-function relationships that underlie these pathological effects remain largely obscure. Here we chose an example mutation (R58Q) in the myosin regulatory light chain (RLC) that is associated with a severe HCM phenotype and combined the results from a wide range of in vitro and in situ structural and functional studies on isolated protein components, myofibrils and ventricular trabeculae to create an extensive map of structure-function relationships.

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Feb
2018

Cardiac myosin binding protein-C (cMyBP-C) is a functional sarcomeric protein that regulates contractility in response to contractile demand, and many mutations in cMyBP-C lead to hypertrophic cardiomyopathy (HCM). To gain insight into the effects of disease-causing cMyBP-C missense mutations on contractile function, we expressed the pathogenic W792R mutation in mouse cardiomyocytes lacking endogenous cMyBP-C and studied the functional effects using three-dimensional engineered cardiac tissue (mECT) constructs. Based on complete conservation of tryptophan at this location in FnIII domains, we hypothesized that the W792R mutation affects folding of the C6 FnIII-domain, destabilizing the mutant protein.

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Feb
2018

Limited medical options are available for rhythm control in patients with atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM). There are no published reports of dofetilide use in this population.
A retrospective chart review was conducted on1,404 patients loaded on dofetilide for AF suppression at the Cleveland Clinic from 2008-2012, 25 of whom were found to have HCM.

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Feb
2018

Hypertrophic cardiomyopathy (HCM) is the most commonly inherited cardiac disease-recent studies suggest a prevalence as high as 1 in 200. For symptomatic patients with obstructive HCM who are refractory to medical therapy, septal reduction is indicated. Septal myectomy (SM) is considered the gold standard septal reduction technique.

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Feb
2018

A 5-month-old intact female Scottish Fold cat was presented for cardiac evaluation. Careful auscultation detected a slight systolic murmur (Levine I/VI). The findings of electrocardiography, thoracic radiography, non-invasive blood pressure measurements and conventional echocardiographic studies were unremarkable.

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Feb
2018

This study aims to assess the impact of morphological type on the prognosis of obstructive hypertrophic cardiomyopathy patients who underwent myectomy.
We recruited 469 obstructive hypertrophic cardiomyopathy patients refractory to medicinal treatment who have undergone surgical myectomy at a nationwide referral centre. All patients were divided into 3 groups based on the morphological classification of left ventricular hypertrophy and were followed up by telephone every year.

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Feb
2018

Hypertrophic cardiomyopathy (HCM) is a serious monogenic disease characterized by cardiac hypertrophy, fibrosis, sudden cardiac death and heart failure. Previously, we identified that miR-139-5p was downregulated in HCM patients. However, the regulatory effects of miR-139-5p remain unclear.

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Feb
2018

Mutations of α-actin gene (ACTC1) have been phenotypically related to various cardiac anomalies, including hypertrophic cardiomyopathy and dilated cardiomyopathy and left ventricular (LV) myocardial noncompaction. A novel ACTC mutation is reported as cosegregating for familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts.
In an Italian family of 7 subjects, 4 aged 10 (II-1), 14 (II-2), 43 (I-4) and 46 years (I-5), presenting abnormal ECG changes, dyspnea and palpitation (II-2, I-4, and I-5), and recurrent cerebral ischemic attack (I-5), underwent 2-dimensional echo, cardiac magnetic resonance, Holter monitoring, and next-generation sequencing gene analysis.

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Feb
2018

Hypertrophic cardiomyopathy is the most common genetic cardiovascular disorder and is associated with symptoms of heart failure and increased risk of sudden cardiac death. The most common condition is obstruction of the left ventricular outflow tract. Surgical septal myectomy and alcohol septal ablation are the 2 accepted modes of septal reduction therapy and are indicated when there are advanced symptoms and a peak left ventricular outflow gradient ≥50 mmHg.

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Feb
2018

Aquaporin-4, encoded by, is the major water channel in the central nervous system and plays an important role in the brain's water balance, including edema formation and clearance. Using genomic copy-number analysis and trio-exome sequencing, we investigated a male patient with intellectual disability, hearing loss, and progressive gait dysfunction and found a de novo missense change Ser111Thr inas the only suspicious finding. Perinatally, signs of brain ischemia were detected in relation to acute collapse 2 h after birth that resolved a few days later.

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Feb
2018

Hypertrophiccardiomyopathy (HCM) is common, whereas the decision not to have an implantable cardioverterdefibrillator (ICD) when probably falling into a 'high-risk' category is not. A solicitor aged 45 years attended the inherited cardiac conditions clinic for review of her HCM and discussion about ICD implantation for primary prevention of sudden cardiac death (SCD). Despite a predicted 7% risk of SCD within the next 5 years, according to the European Society of Cardiology endorsed HCM Risk-SCD risk stratification tool, the patient opted against implantation of an ICD and comprehensively justifies her decision.

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Feb
2018

Infantile-onset Pompe disease is a kind of glycogenosis resulting from a deficit of the enzyme acid alpha-glucosidase. Before specific enzyme replacement therapy (ERT) became available, the classic form was fatal during the first two years of life. ERT increases survival and improves cardiac, respiratory and motor functioning.

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Feb
2018

Interventional techniques have been developed for a wide spectrum of mechanisms of heart failure (HF), especially in valvular heart disease and cardiomyopathies (ischaemic cardiomyopathy and hypertrophic cardiomyopathy). In this article, we review recent reports on catheter interventions to treat patients with HF.
Direct modification using the Parachute device and the REVIVENT-TC device for patients with impaired left ventricle with large infarct scars improves geometry and haemodynamic efficiency, resulting in a reduction of HF symptoms.

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Nov
2017

Cardiomyopathy: An Overview.

Am Fam Physician 2017 Nov;96(10):640-646
Jay Brieler, Matthew A Breeden, Jane Tucker
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death.

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Feb
2018

Exercise intolerance is the most common symptom in hypertrophic cardiomyopathy (HCM). We examined whether inability to augment myocardial mechanics during exercise would influence functional performance and clinical outcomes in HCM.
Ninety-five HCM patients (32 nonobstructive, 32 labile-obstructive, 31 obstructive) and 26 controls of similar age and gender distribution were recruited prospectively.

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Mar
2018

Ventricular Assist Devices for Neonates and Infants.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2018 Mar;21:9-14
Katsuhide Maeda, David N Rosenthal, Olaf Reinhartz
Heart transplant waitlist survival in pediatric patients has been substantially improved since the introduction of pediatric-specific ventricular assist device. In neonates and infants, however, the waitlist mortality remains very high. The only long-term device currently approved for use in the United States is the Berlin Heart EXCOR, but this device has several important limitations because of the paracorporeal, pulsatile nature of the underlying technology.

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Feb
2018

Fibroblast growth factor 23 (FGF23) is discussed as a new biomarker of cardiac hypertrophy and mortality in patients with and without chronic kidney disease (CKD). We previously demonstrated that FGF23 is expressed by cardiac myocytes, enhanced in CKD and induces cardiac hypertrophy via activation of FGF receptor 4 independent of its co-receptor klotho. The impact of FGF23 on cardiac fibrosis is largely unknown.

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Feb
2018

The following information is missing from the Funding footnote on the first page of the published article: "This study was partly funded by NIH RO1 HL092985." The last/corresponding author is incorrectly listed on the first page of the published article: The correct name is Abraham MR.

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Jan
2018

Subaortic septal myectomy is usually performed to mitigate obstruction in patients with the obstructive form of hypertrophic cardiomyopathy (HCM) or in those with congenital subaortic stenosis. Moreover, it is combined with aortic valve replacement in patients with severe aortic valve stenosis (SAS) and asymmetrical septal hypertrophy causing concomitant left ventricular outflow tract obstruction. When both conditions coexist, it is conceptually difficult to identify a cardiomyopathy beyond an adaptive myocardial hypertrophy, strictly related to pressure overload.

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Jan
2018

This study aims to review the influence of gender-specific differences and patient demographics on cardiac device and pacing mode selection over a 10-year period.
We retrospectively reviewed patients who underwent first implantation of the cardiac device between January 1, 2006 and June 31, 2016.
During the study period, 704 patients underwent first cardiac device implantation.

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Dec
1969

Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited.
Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection.
This retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution.

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Mar
2018

Sudden cardiac death (SCD) risk stratification in hypertrophic cardiomyopathy (HCM) in the context of primary prevention remains suboptimal. The purpose of this study was to examine the additional contribution of programmed ventricular stimulation (PVS) on established risk assessment.
Two-hundred-and-three consecutive patients with diagnosed HCM and ≥1 noninvasive risk factors were prospectively enrolled over 19years.

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Mar
2018

Coronary vascular dysfunction is linked with poor cardiovascular prognosis in patients without obstructive coronary artery disease (CAD) but a critical appraisal of the literature is lacking.
We performed a systematic review and meta-analysis to quantify the cardiovascular risk associated with endothelial dependent and non-endothelial dependent coronary vascular dysfunction in patients with normal or non-obstructive CAD (epicardial stenosis <50%). Prospective cohort studies that reported coronary vascular dysfunction at baseline and cardiovascular outcomes at follow-up were included.

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Feb
2018

To investigate the association between serum omentin-1 levels and adverse cardiac events in patients with hypertrophic cardiomyopathy (HCM).
This prospective observational study included 87 patients with HCM and 50 age- and sex-matched control subjects. Serum omentin-1 and brain natriuretic peptide (BNP) levels were measured in all the subjects using enzyme-linked immunosorbent assay and electrochemiluminescence methods, respectively.

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Feb
2018

Cardiac Troponin T (TnT) mutation-linked familial hypertrophic cardiomyopathy (FHC) is known to cause sudden cardiac death at a young age. Here, we investigated the role of the Carelease channel of the cardiac sarcoplasmic reticulum (SR), ryanodine receptor (RyR2), in the pathogenic mechanism of lethal arrhythmia in FHC-related TnT-mutated transgenic mice (TG; TnT-delta160E).
In TG cardiomyocytes, the Caspark frequency (SpF) was much higher than that in non-TG cardiomyocytes.

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