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Pubblicazioni recenti - dilated cardiomyopathy
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Non-heart transplant surgical approaches with mitral valve operation and surgical ventricular reconstruction for non-ischaemic dilated cardiomyopathy: a Japanese multicenter study.
Gen Thorac Cardiovasc Surg2020 Oct;():. doi: 10.1007/s11748-020-01512-1.
Cho Yasunori, Wakasa Satoru, Usui Akihiko, Minatoya Kenji, Arai Hirokuni, Yaku Hitoshi, Yamaguchi Atsushi, Komiya Tatsuhiko, Matsumiya Goro, Hamano Kimikazu, Saiki Yoshikatsu, Matsui Yoshiro,
Abstract
OBJECTIVES:
There is uncertainty over the efficacy of additional surgical ventricular reconstruction (SVR) associated with mitral valve operation for non-ischaemic dilated cardiomyopathy (DCM). This study aims to assess mid-term outcomes of these non-heart transplant surgical approaches for DCM.
METHODS:
We reviewed retrospectively 106 patients (median age 64, 44 females) who underwent isolated mitral annular plasty (MAP; n?=?34), mitral valve replacement (MVR; n?=?29), and SVR associated with MAP (SVR?+?MAP; n?=?43) for DCM, in 11 Japanese hospitals. We analysed mid-term outcomes, specifically freedom from cardiac death and cardiac event.
RESULTS:
Hospital deaths occurred in 16 patients (15.1%) and cardiac deaths in 36 patients (34.0%) during the study period of 4.4?±?3.5 years. Freedom from cardiac death at 7 years in patients undergoing MAP, MVR, and SVR?+?MAP were, respectively, 79.1%, 82.6%, and 29.5% (P?
CONCLUSIONS:
This study could not show any benefit of additional SVR, by means of volume reduction, to MAP, because the baseline characteristics were different even after the stratification of DCM grade. MVR can be performed with favorable mid-term outcomes even in patients with advanced DCM, while patients undergoing MAP with/without SVR had more frequent MR recurrence or cardiac events. Interestingly, the right ventricular feature is a predictor of both cardiac death and events, with the TR grade being a predictor of poor mid-term outcomes.
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Functional significance of intra-left ventricular vortices on energy efficiency in normal, dilated, and hypertrophied hearts.
J Clin Ultrasound2020 Oct;():. doi: 10.1002/jcu.22938.
Sarashina-Motoi Miwa, Iwano Hiroyuki, Motoi Ko, Ishizaka Suguru, Chiba Yasuyuki, Tsujinaga Shingo, Murayama Michito, Nakabachi Masahiro, Yokoyama Shinobu, Nishino Hisao, Okada Kazunori, Kaga Sanae, Anzai Toshihisa,
Abstract
PURPOSE:
To investigate the influence of changes in vortices within the left ventricle (LV) on energy efficiency (EE) in normal and diseased hearts.
METHODS:
We performed vector flow mapping echocardiography in 36 normal participants (N), 36 patients with dilated cardiomyopathy (D), and 36 patients with LV hypertrophy (H). The circulation of the main anterior vortex was measured as a parameter of vortex strength. Energy loss (EL) was measured for one cardiac cycle, and EE was calculated as EL divided by stroke work (SW), which represents the loss of kinetic energy per unit of LV external work.
RESULTS:
Circulation increased in the order of N, H, and D (N: 15?±?4, D: 19?±?8, H: 17?±?6?×?10 m /s; analysis of variance [ANOVA] P?
CONCLUSIONS:
Enhanced vortices could be associated with effective increase in LV external work in normal hearts. Conversely, they were associated with loss of EE without an optimal increase in external work in failing hearts, regardless of the LV morphology.
© 2020 Wiley Periodicals LLC.
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Tafazzin Mutation Affecting Cardiolipin Leads to Increased Mitochondrial Superoxide Anions and Mitophagy Inhibition in Barth Syndrome.
Cells2020 Oct;9(10):. doi: E2333.
Petit Patrice X, Ardilla-Osorio Hector, Penalvia Lucile, Nathan E Rainey,
Abstract
Tafazzin is a phospholipid transacylase that catalyzes the remodeling of cardiolipin, a mitochondrial phospholipid required for oxidative phosphorylation. Mutations of the tafazzin gene cause Barth syndrome, which is characterized by mitochondrial dysfunction and dilated cardiomyopathy, leading to premature death. However, the molecular mechanisms underlying the cause of mitochondrial dysfunction in Barth syndrome remain poorly understood. We again highlight the fact that the tafazzin deficiency is also linked to defective oxidative phosphorylation associated with oxidative stress. All the mitochondrial events are positioned in a context where mitophagy is a key element in mitochondrial quality control. Here, we investigated the role of tafazzin in mitochondrial homeostasis dysregulation and mitophagy alteration. Using a HeLa cell model of tafazzin deficiency, we show that dysregulation of tafazzin in HeLa cells induces alteration of mitophagy. Our findings provide some additional insights into mitochondrial dysfunction associated with Barth syndrome, but also show that mitophagy inhibition is concomitant with apoptosis dysfunction through the inability of abnormal mitochondrial cardiolipin to assume its role in cytoplasmic signal transduction. Our work raises hope that pharmacological manipulation of the mitophagic pathway together with mitochondrially targeted antioxidants may provide new insights leading to promising treatment for these highly lethal conditions.
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LncRNA AC061961.2 overexpression inhibited endoplasmic reticulum stress induced apoptosis in dilated cardiomyopathy rats and cardiomyocytes via activating wnt/?-catenin pathway.
J Recept Signal Transduct Res2020 Oct;():1-10. doi: 10.1080/10799893.2020.1828915.
Qiu Zhibing, Chen Wen, Liu Yafeng, Jiang Ben, Yin Li, Chen Xin,
Abstract
Down-regulated lncRNA AC061961.2 in dilated cardiomyopathy (DCM) patients was previous reported. Whether AC061961.2 has regulatory effect on DCM still need exploration. Here, we tried to investigate the effect of AC061961.2 on DCM. After DCM model rat was established through injecting Adriamycin, left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), left ventricular ejection fraction (LVEF), and left ventricular fractional shortening (LVFS) were measured by echocardiography. Histopathological changes and apoptosis were detected by hematoxylin-eosin, Masson staining, and TUNEL. After cardiomyocytes were isolated and identified by immunofluorescence, DCM cell model was established by injecting adriamycin. After transfected with overexpressed-AC061961.2 plasmids, cell apoptosis was detected by flow cytometry. The expressions of AC061961.2, ?-catenin, Axin2, c-Myc, CRP78, CHOP, Caspase-3, Bcl-2, and Bax in cardiomyocytes and heart tissues were detected by RT-qPCR or western blot. LVEDD and LVESD were increased while LVEF and LVFS were decreased in DCM rats. The histopathological of heart tissues showed a typical sign of DCM. Apoptosis were increased in heart tissues of DCM rats. In DCM rats, the expressions of AC061961.2, ?-catenin, Axin2, c-Myc, and Bcl-2 were decreased, the expressions of CRP78, CHOP, Caspase-3, and Bax were increased. After the overexpression of AC061961.2, levels of ?-catenin, Axin2, c-Myc, and Bcl-2 were increased, while levels of CRP78, CHOP, Caspase-3, and Bax were decreased, compared with that in DCM cardiomyocytes. LncRNA AC061961.2 overexpression inhibited endoplasmic reticulum stress induced apoptosis in DCM rats and cardiomyocytes activating Wnt/?-catenin pathway.
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Endocardial fibroelastosis in a dog with congestive heart failure.
J Vet Cardiol2020 Sep;32():33-39. doi: S1760-2734(20)30079-5.
Schreiber N, Baron Toaldo M, Romero-Palomo F, Sydler T, Glaus T,
Abstract
In a 6-month-old, intact female, Japanese spitz presenting with severe dyspnea, lung ultrasonography revealed confluent B lines associated with severe echocardiographic left sided volume overload and systolic dysfunction. A congenital shunt or valvular dysplasia was not demonstrable. On electrocardiogram, there was a constant sinus rhythm, respectively sinus tachycardia. Cardiac troponin I was normal. Within 2 days of admission, the dog died of heart failure. On macroscopic postmortem examination, the left ventricle and atrium were markedly dilated, and the left ventricular endocardium had a mild diffuse whitish appearance. Histopathology revealed moderate to severe thickening of the left ventricular endocardium, composed mostly of abundant elastic fibers and fewer collagen fibers, diagnostic for endocardial fibroelastosis. In addition, there were mild degenerative changes of the atrioventricular valves. Endocardial fibroelastosis is a rare congenital disease and should be considered in a young dog if more common causes of echocardiographic dilated cardiomyopathy phenotype are ruled out.
Copyright © 2020 Elsevier B.V. All rights reserved.
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Cardiac sequelae of novel coronavirus disease 2019 (COVID-19): a clinical case series.
Eur Heart J Case Rep2020 Oct;4(FI1):1-6. doi: 10.1093/ehjcr/ytaa179.
Demertzis Zachary D, Dagher Carina, Malette Kelly M, Fadel Raef A, Bradley Patrick B, Brar Indira, Rabbani Bobak T, Suleyman Geehan,
Abstract
Background:
COVID-19 caused by severe acute respiratory syndrome coronavirus 2 most commonly manifests with fever and respiratory illness. The cardiovascular manifestations have become more prevalent but can potentially go unrecognized. We look to describe cardiac manifestations in three patients with COVID-19 using cardiac enzymes, electrocardiograms, and echocardiography.
Case summaries:
The first patient, a 67-year-old Caucasian female with non-ischaemic dilated cardiomyopathy, presented with dyspnoea on exertion and orthopnoea 1 week after testing positive for COVID-19. Echocardiogram revealed large pericardial effusion with findings consistent with tamponade. A pericardial drain was placed, and fluid studies were consistent with viral pericarditis, treated with colchicine, hydroxychloroquine, and methylprednisolone. Follow-up echocardiograms showed apical hypokinesis, that later resolved, consistent with Takotsubo syndrome. The second patient, a 46-year-old African American male with obesity and type 2 diabetes mellitus presented with fevers, cough, and dyspnoea due to COVID-19. Clinical course was complicated with pulseless electrical activity arrest; he was found to have D-dimer and troponin elevation, and inferior wall ST elevation on ECG concerning for STEMI due to microemboli. The patient succumbed to the illness. The third patient, a 76-year-old African American female with hypertension, presented with diarrhoea, fever, and myalgia, and was found to be COVID-19 positive. Clinical course was complicated, with acute troponin elevation, decreased cardiac index, and severe hypokinesis of the basilar wall suggestive of reverse Takotsubo syndrome. The cardiac index improved after pronation and non-STEMI therapy; however, the patient expired due to worsening respiratory status.
Discussion:
These case reports demonstrate cardiovascular manifestations of COVID-19 that required monitoring and urgent intervention.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Myocarditis Associated With COVID-19.
Am J Med Case Rep2020 ;8(12):498-502. doi: 10.12691/ajmcr-8-12-19.
Oleszak Filip, Maryniak Andrii, Botti Evan, Abrahim Christian, Salifu Moro O, Youssef Mary, Henglein Victoria L, McFarlane Samy I,
Abstract
Coronavirus disease 2019 (COVID-19) has rapidly evolved into a global pandemic, with affecting to-date over 23 million people and causing over 800,000 deaths around the globe. The major pathogenetic mechanisms include inflammation, vasoconstriction and thrombogenesis. Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) typically manifests as fever, cough, shortness of breath, and exhibits radiographic evidence of bilateral pneumonic infiltrates. Recent meta-analyses have shown that myocardial injury, including viral myocarditis, is prevalent among infected patients, especially in patients requiring ICU level care. Diagnosis of viral myocarditis is multifactorial and involves detection of elevated cardiac biomarkers and echocardiographic evidence of cardiomyopathy, in the absence of diseased coronary arteries. Endomyocardial biopsy with histopathologic examination provides definitive confirmation. We present a case of a previously healthy 52-year-old male who presented clinically with suspected myocarditis with new-onset dilated cardiomyopathy (DCM) and systolic dysfunction as a sequela of infection with SARS-CoV-2. In this report we highlight the clinical presentation of echocardiographic findings and proposed pathogenetic mechanisms of myocarditis associated with COVID-19 which has a varied presentation, ranging from clinically silent to life-threatening arrhythmias with hemodynamic compromise.
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Manipulation of the gut microbiota by the use of prebiotic fibre does not override a genetic predisposition to heart failure.
Sci Rep2020 Oct;10(1):17919. doi: 10.1038/s41598-020-73614-y.
Jama Hamdi A, Fiedler April, Tsyganov Kirill, Nelson Erin, Horlock Duncan, Nakai Michael E, Kiriazis Helen, Johnson Chad, Du Xiao-Jun, Mackay Charles R, Marques Francine Z, Kaye David M,
Abstract
Increasing evidence supports a role for the gut microbiota in the development of cardiovascular diseases such as hypertension and its progression to heart failure (HF). Dietary fibre has emerged as a modulator of the gut microbiota, resulting in the release of gut metabolites called short-chain fatty acids (SCFAs), such as acetate. We have shown previously that fibre or acetate can protect against hypertension and heart disease in certain models. HF is also commonly caused by genetic disorders. In this study we investigated whether the intake of fibre or direct supplementation with acetate could attenuate the development of HF in a genetic model of dilated cardiomyopathy (DCM) due to overexpression of the cardiac specific mammalian sterile 20-like kinase (Mst1). Seven-week-old male mice DCM mice and littermate controls (wild-type, C57BL/6) were fed a control diet (with or without supplementation with 200 mM magnesium acetate in drinking water), or a high fibre diet for 7 weeks. We obtained hemodynamic, morphological, flow cytometric and gene expression data. The gut microbiome was characterised by 16S rRNA amplicon sequencing. Fibre intake was associated with a significant shift in the gut microbiome irrespective of mouse genotype. However, neither fibre or supplementation with acetate were able to attenuate cardiac remodelling or cardiomyocyte apoptosis in Mst1 mice. Furthermore, fibre and acetate did not improve echocardiographic or hemodynamic parameters in DCM mice. These data suggest that although fibre modulates the gut microbiome, neither fibre nor acetate can override a strong genetic contribution to the development of heart failure in the Mst1 model.
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A combination of quinidine/mexiletine reduces arrhythmia in dilated cardiomyopathy in two patients with R814W SCN5A mutation.
ESC Heart Fail2020 Oct;():. doi: 10.1002/ehf2.12993.
Zakrzewska-Koperska Joanna, Bili?ska Zofia T, Truszkowska Gra?yna T, Franaszczyk Maria, Elikowski Waldemar, Warmi?ski Grzegorz, Kalin Katarzyna, Urbanek Piotr, Bodalski Robert, Orczykowski Micha?, Szumowski ?ukasz, P?oski Rafa?, Bili?ska Maria,
Abstract
SCN5A gene mutations are described in 2% of patients with dilated cardiomyopathy (DCM) and different rhythm disturbances, including multifocal ectopic Purkinje-related premature contractions. Recent data indicate that sodium channel blockers are particularly effective monotherapy in carriers of the R222Q SCN5A variant. Our purpose is to describe the effectiveness of antiarrhythmic treatment in a family with genetically determined arrhythmogenic DCM associated with the R814W variant in the SCN5A gene. We examined a family with arrhythmogenic DCM (multifocal ectopic Purkinje-related premature contractions phenotype, atrial tachyarrhythmias, automatism, and conduction disorders) and described antiarrhythmic treatment efficacy in heart failure symptoms reduction and myocardial function improvement. We found a heterozygotic mutation R814W in SCN5A by whole exome sequencing in the proband and confirmed its presence in all affected subjects. There were two sudden cardiac deaths and one heart transplantation among first-degree relatives. The 58-year-old father and his 37-year-old daughter had full spectrum of symptoms associated with R814W SCN5A mutation. Both had implanted cardioverter defibrillator. In the father, adding mexiletine to quinidine therapy reduced ventricular arrhythmia (50-60% ? 6-8% of whole rhythm) and reverted long-standing atrial fibrillation to sinus rhythm. In the daughter, mexiletine and overdrive pacing were effective in ventricular arrhythmia reduction (25% ? 0.01%). Because of a growing number of atrial fibrillation recurrences, a reduced dose of quinidine (subsequently flecainide) was added, resulting in arrhythmia significant reduction. In both cases, antiarrhythmic effectiveness correlated with clinical improvement. In SCN5A R814W-associated DCM, a combination of Class I antiarrhythmics and overdrive pacing is an effective treatment of severe ventricular and atrial arrhythmias.
© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.
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Genomic testing in 1019 individuals from 349 Pakistani families results in high diagnostic yield and clinical utility.
NPJ Genom Med2020 ;5():44. doi: 10.1038/s41525-020-00150-z.
Cheema Huma, Bertoli-Avella Aida M, Skrahina Volha, Anjum Muhammad Nadeem, Waheed Nadia, Saeed Anjum, Beetz Christian, Perez-Lopez Jordi, Rocha Maria Eugenia, Alawbathani Salem, Pereira Catarina, Hovakimyan Marina, Patric Irene Rosita Pia, Paknia Omid, Ameziane Najim, Cozma Claudia, Bauer Peter, Rolfs Arndt,
Abstract
We implemented a collaborative diagnostic program in Lahore (Pakistan) aiming to establish the genetic diagnosis, and to asses diagnostic yield and clinical impact in patients with suspected genetic diseases. Local physicians ascertained pediatric patients who had no previous access to genetic testing. More than 1586 genetic tests were performed in 1019 individuals (349 index cases, 670 relatives). Most frequently performed tests were exome/genome sequencing (ES/GS, 284/78 index cases) and specific gene panels (55 index cases). In 61.3% of the patients (?=?214) a genetic diagnosis was established based on pathogenic and likely pathogenic variants. Diagnostic yield was higher in consanguineous families (60.1 vs. 39.5%). In 27 patients, genetic diagnosis relied on additional biochemical testing, allowing rapid assessment of the functional effect of the variants. Remarkably, the genetic diagnosis had a direct impact on clinical management. Most relevant consequences were therapy related such as initiation of the appropriated treatment in a timely manner in 51.9% of the patients (?=?111). Finally, we report 12 candidate genes among 66 cases with no genetic diagnosis. Importantly, three of these genes were validated as 'diagnostic' genes given the strong evidence supporting causality derived from our data repository dilated cardiomyopathyintellectual disability and liver cholestasis). The high diagnostic yield, clinical impact, and research findings demonstrate the utility of genomic testing, especially when used as first-line genetic test. For patients with suspected genetic diseases from resource-limited regions, ES can be considered as the test of choice to achieve genetic diagnosis.
© The Author(s) 2020.
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Sudden Cardiac Death in the Young: Incidence, Trends, and Risk Factors in a Nationwide Study.
Circ Cardiovasc Qual Outcomes2020 Oct;13(10):e006470. doi: 10.1161/CIRCOUTCOMES.119.006470.
Ha Francis J, Han Hui-Chen, Sanders Prashanthan, Fendel Kim, Teh Andrew W, Kalman Jonathan M, O'Donnell David, Leong Trishe, Farouque Omar, Lim Han S,
Abstract
BACKGROUND:
Sudden cardiac death (SCD) in the young is devastating. Contemporary incidence remains unclear with few recent nationwide studies and limited data addressing risk factors for causes. We aimed to determine incidence, trends, causes, and risk factors for SCD in the young.
METHODS AND RESULTS:
The National Coronial Information System registry was reviewed for SCD in people aged 1 to 35 years from 2000 to 2016 in Australia. Subjects were identified by the , code relating to circulatory system diseases (I00-I99) from coronial reports. Baseline demographics, circumstances, and cause of SCD were obtained from coronial and police reports, alongside autopsy and toxicology analyses where available. During the study period, 2006 cases were identified (median age, 28±7 years; men, 75%; mean body mass index, 29±8 kg/m). Annual incidence ranged from 0.91 to 1.48 per 100?000 age-specific person-years, which was the lowest in 2013 to 2015 compared with previous 3-year intervals on Poisson regression model (=0.001). SCD incidence was higher in nonmetropolitan versus metropolitan areas (0.99 versus 0.53 per 100?000 person-years; <0.001). The most common cause of SCD was coronary artery disease (40%), followed by sudden arrhythmic death syndrome (14%). Incidence of coronary artery disease-related SCD decreased from 2001-2003 to 2013-2015 (<0.001). Proportion of SCD related to sudden arrhythmic death syndrome increased during the study period (=0.02) although overall incidence was stable (=0.22). Residential remoteness was associated with coronary artery disease-related SCD (odds ratio, 1.44 [95% CI, 1.24-1.67]; <0.001). For every 1-unit increase, body mass index was associated with increased likelihood of SCD from cardiomegaly (odds ratio, 1.08 [95% CI, 1.05-1.11]; <0.001) and dilated cardiomyopathy (odds ratio, 1.04 [95% CI, 1.01-1.06]; =0.005).
CONCLUSIONS:
Incidence of SCD in the young and specifically coronary artery disease-related SCD has declined in recent years. Proportion of SCD related to sudden arrhythmic death syndrome increased over the study period. Geographic remoteness and obesity are risk factors for specific causes of SCD in the young.
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Prevalence of Chagas heart disease in dilated cardiomyopathy.
Arch Cardiol Mex2020 Oct;91(4):. doi: 10.24875/ACM.20000042.
González-Zambrano Héctor, Amaya-Tapia Gerardo, Franco-Ramos María C, López León-Murguía Oscar J,
Abstract
OBJECTIVES:
The main objective is to determine the prevalence of American trypanosomiasis in patients with dilated cardiomyopathy in a tertiary hospital in western Mexico.
METHODS:
From January 1991 to February 2016, 387 consecutive patients with a confirmed diagnosis of dilated cardiomyopathy were included in the study. Cases with ventricular dilatation secondary to ischemic heart disease, valvular heart disease, hypertension, lung disease, pericardial disease, or congenital heart disease were excluded from the study. Diagnosis was made detecting antibodies against Trypanosoma cruzi with two different methods or parasite in blood.
RESULTS:
Were included 387 patients with dilated cardiomyopathy, Chagas cardiomyopathy was confirmed in 6.9%, two patients in the acute phase (in one, suspected transfusion transmission was detected). Most patients were born in rural areas. About 96.2% showed congestive heart failure, only one patient with apical left ventricular aneurysm manifested palpitations. About 66% with right bundle branch block, left anterior fascicular block, or the association of both, in 14.8%, non-sustained ventricular tachycardia was found.
CONCLUSIONS:
Chagas cardiomyopathy is common in México, mainly in people who were born or lived during childhood in rural areas. It is a common cause of heart failure. Chagas' heart disease should be suspected in patients receiving a blood transfusion, even without another epidemiological history.
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Preclinical and pharmacokinetic properties of danicamtiv, a new targeted myosin activator for the treatment of dilated cardiomyopathy.
Xenobiotica2020 Oct;():1-58. doi: 10.1080/00498254.2020.1839982.
Grillo Mark P, Markova Svetlana, Evanchik Marc, Trellu Marc, Moliner Patricia, Brun Priscilla, Perreard-Dumaine Anne, Vicat Pascale, Driscoll Jim, Carlson Tim J,
Abstract
Dilated cardiomyopathy (DCM) is a disease of the myocardium defined by left ventricular enlargement and systolic dysfunction leading to heart failure. Danicamtiv, a new targeted myosin activator designed for the treatment of DCM, was characterized in and preclinical studies. Danicamtiv human hepatic clearance was predicted to be 0.5?mL/min/kg from metabolic stability studies in human hepatocytes. For human, plasma protein binding was moderate with a fraction unbound of 0.16, whole blood-to-plasma partitioning ratio was 0.8, and danicamtiv showed high permeability and no efflux in a Caco-2 cell line. Danicamtiv metabolism pathways included CYP-mediated amide-cleavage, -demethylation, as well as isoxazole- and piperidine-ring-opening. Danicamtiv clearance was low across species with 15.5, 15.3, 1.6, and 5.7?mL/min/kg in mouse, rat, dog, and monkey, respectively. Volume of distribution ranged from 0.24?L/kg in mouse to 1.7?L/kg in rat. Oral bioavailability ranged from 26% in mouse to 108% in dog. Simple allometric scaling prediction of human plasma clearance, volume of distribution, and half-life was 0.64?mL/min/kg, 0.98?L/kg, and 17.7?h, respectively. Danicamtiv preclinical attributes and predicted human pharmacokinetics supported advancement toward clinical development.
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Transvenous retrieval of a pulmonary artery catheter knot around pacing leads: A case report.
Cardiovasc Revasc Med2020 Sep;():. doi: S1553-8389(20)30589-3.
Egbuche Obiora, Nwagbara Kenechukwu, Mezue Kenechukwu N, Abe Temidayo, Nwokike Shirley,
Abstract
BACKGROUND:
Pulmonary Artery Catheter (PAC) knotting is a rare complication of PAC insertion. In patients with dilated right heart chambers, blind insertion of PAC significantly increases the risk of catheter knotting. We demonstrate a safe and successful approach to resolving a PAC knot around pacing leads of a cardiac resynchronization device.
CASE PRESENTATION:
A 63-year-old African American male with dilated cardiomyopathy and a cardiac resynchronization therapy (CRT) device for severe left ventricular systolic dysfunction required PAC insertion for hemodynamic management of acute heart failure. PAC insertion was complicated by catheter knotting around the pacing leads. The PAC was successfully retrieved using a transvenous technique.
CONCLUSION:
Fluoroscopy-guided insertion of PAC is advisable and preferred over blind insertion in patients with high risk of PAC entanglement.
LEARNING OBJECTIVE:
To highlight a potential complication of blind pulmonary artery catheter insertion and provide a safe technique to resolve catheter knots.
Copyright © 2020 Elsevier Inc. All rights reserved.
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[Becker dystrophy induced familial dilated cardiomyopathy: two cases report].
Zhonghua Xin Xue Guan Bing Za Zhi2020 Oct;48(10):873-875. doi: 10.3760/cma.j.cn112148-20191231-00784.
Long Y, Man Q S, Li X, Li Y, Feng X,
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Analysis of DCM associated protein alterations of human right and left ventricles.
J Proteomics2020 Oct;():104018. doi: S1874-3919(20)30386-9.
Ameling Sabine, Bischof Julia, Dörr Marcus, Könemann Stephanie, Empen Klaus, Weitmann Kerstin, Klingel Karin, Beug Daniel, Dhople Vishnu Mukund, Völker Uwe, Hammer Elke, Felix Stephan B,
Abstract
Dilated cardiomyopathy (DCM) is characterized by ventricular chamber enlargement and impaired myocardial function. Endomyocardial biopsies (EMB) enable immunohistochemical and molecular characterization of this disease. However, knowledge about specific molecular patterns and their relation to cardiac function in both ventricles is rare. Therefore, we performed a mass spectrometric analysis of 28 paired EMBs of left (LV) and right ventricles (RV) of patients with DCM or suspected myocarditis allowing quantitative profiling of 743 proteins. We analysed associations between protein abundance of LV and RV as well as the echocardiographic parameters LVEF, TAPSE, LVEDDI, and RVEDDI by linear regression models. Overall, more LV than RV proteins were associated with LV parameters or with RVEDDI. Most LV and RV proteins increasing in level with impairing of LVEF were annotated to structural components of cardiac tissue. Additionally, a high proportion of LV proteins with metabolic functions decreased in level with decreasing LVEF. Results were validated with LV heart sections of a genetic murine heart failure model. The study shows, that remodelling and systolic dysfunction in DCM is mirrored by distinct alterations in protein composition of both ventricles. Loss of LV systolic function is reflected predominantly by alterations in proteins assigned to metabolic functions in the LV whereas structural remodelling was more obvious in the RV. Alterations related to intermediate filaments were seen in both ventricles and highlight such proteins as early indicators of LV loss of function. SIGNIFICANCE: The present study report protein sets in the RV and the LV being associated with ventricular function and remodelling in DCM. Protein abundances in the LV and the RV emphasize and expand current knowledge on pathophysiological changes in heart failure and DCM. While RV and LV EMBs do not differ concerning diagnostic assessment of inflammatory status and virus persistence, additional information reflecting disease severity associated protein alterations can be gained by EMB protein profiling. RV and LV protein data provided complementary information. The protein pattern of the LV reflects metabolic changes and an impaired energy production, which is associated with the degree of LV systolic dysfunction and remodelling and may yield important information about the disease status in DCM. On the other hand, at this disease stage of DCM with still preserved RV function, RV alterations in structural proteins may reflect myocardial compensatory protective mechanisms for maintenance of structure and cellular function. The study highlight particular proteins being of interest as heart failure biomarkers in both ventricles which seem to reflect the severity of the disease. Further comparative studies between different HF aetiologies have to evaluate those proteins as markers specific for DCM.
Copyright © 2019. Published by Elsevier B.V.
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Nonimmune hydrops fetalis management from the perspective of fetal cardiologists: A single tertiary center experience from Egypt.
J Neonatal Perinatal Med2020 Oct;():. doi: 10.3233/NPM-200491.
Rakha S, Elmarsafawy H,
Abstract
BACKGROUND:
Despite advances in managing nonimmune hydrops fetalis (NIHF), perinatal mortality is still significant. Fetal cardiac failure eventually occurs regardless of etiology. However, no previous study has addressed NIHF from fetal cardiologists' perspective. Therefore, we evaluated etiology and management of a NIHF cohort requiring fetal cardiologist consultation in a developing country.
METHODS:
A single-center retrospective cohort study of 70 cases with NIHF that were referred to a fetal cardiology unit over four years was performed. Demographics, etiologic diagnosis, and outcomes of the cases were assessed. Antenatal management was evaluated using cardiovascular profile score (CVPS).
RESULTS:
The most frequent diagnosis was Idiopathic hydrops 42(62.6%), followed by hydrops due to cardiac diseases 19(28.4%), and 3 dead fetuses were detected at the first fetal echocardiography. Treatment of fetal tachyarrhythmia (n?=?7) had 100% success rate in terms of antenatal hydrops resolution. Digoxin was used in cases of structural heart diseases, twin- twin transfusion syndrome, and dilated cardiomyopathy with perinatal mortality occurring in all cases (n?=?9). In cases of idiopathic hydrops, 14 fetuses received digoxin with intrauterine hydrops resolution in 2/14 (14%) while non-treated cases had intrauterine or early neonatal death.
CONCLUSION:
Nonimmune hydrops is the worst complication of diverse etiologies. Limitations in resources for advanced investigations in developing countries increase the possibility of categorizing NIHF as idiopathic. Tachyarrhythmia induced hydrops can be entirely reversed with antenatal therapy while non-tachyarrhythmia fetal cardiac disease outcomes are unfavorable regardless of therapy. On the other hand, idiopathic hydrops shows a limited potential response to digoxin in utero.
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Early- and late anthracycline-induced cardiac dysfunction: echocardiographic characterization and response to heart failure therapy.
Cardiooncology2020 ;6():23. doi: 10.1186/s40959-020-00079-3.
Kamphuis Janine A M, Linschoten Marijke, Cramer Maarten J, Doevendans Pieter A, Asselbergs Folkert W, Teske Arco J,
Abstract
Background:
Anthracycline-induced cardiac dysfunction (ACD) is a notorious side effect of anticancer treatment. It has been described as a phenomenon of a continuous progressive decline of cardiac function, eventually leading to dilated cardiomyopathy (DCM). This progressive nature suggests that patients with a delayed ACD diagnosis have greater compromise of cardiac function and more adverse remodeling, with a poor response to heart failure (HF) treatment. This study aimed to delineate the impact of a delayed ACD diagnosis on echocardiographic characteristics and response to HF treatment.
Methods and results:
From the population of our cardio-oncology outpatient clinic, 92 ACD patients were included in this study (age 51.6?±?16.2?years, median cumulative anthracycline dose 329 [200-329] mg/m), and a median follow-up of 25.0 [9.6-37.2] months after ACD diagnosis. Median time to ACD diagnosis for patients diagnosed early (?1?year) was 4.0 vs. 47.7?months respectively. There were no echocardiographic differences between patients diagnosed early vs. late (LVEF 43.6?±?4.9% vs. 43.0?±?6.2% and iEDV 63.6 vs. 62.9?mL/m). Eighty-three percent of patients presented with mild LV dysfunction and in 79% the LV was not dilated. Patients diagnosed early were more likely to have (partial) recovery of cardiac function upon HF treatment initiation (?=?0.015).
Conclusions:
In the setting of a cardio-oncology outpatient clinic, patients with ACD presented with a hypokinetic non-dilated cardiomyopathy, rather than typical DCM. Timing of ACD diagnosis did not impact HF disease severity. However, in patients receiving an early diagnosis, cardiac function was more likely to recover upon HF treatment.
© The Author(s) 2020.
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Variability in clinical decision-making for ventricular assist device implantation in pediatrics.
Pediatr Transplant2020 Oct;():e13840. doi: 10.1111/petr.13840.
Joong Anna, Gossett Jeffrey G, Blume Elizabeth D, Thrush Philip, Pahl Elfriede, Mongé Michael C, Backer Carl L, Patel Angira,
Abstract
BACKGROUND:
Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making.
METHODS:
An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed.
RESULTS:
Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications.
CONCLUSIONS:
Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences.
© 2020 Wiley Periodicals LLC.
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Infections persistantes à entérovirus et pathologies humaines.
Virologie (Montrouge)2014 Dec;18(6):306-324. doi: 10.1684/vir.2014.0582.
Renois Fanny, Bouin Alexis, Wehbe Michel, Leveque Nicolas, Andreoletti Laurent,
Abstract
Enteroviruses (EVs) are small naked single-stranded positive RNA viruses (Picornaviridae) of approximately 7,400 nucleotides divided in four species (HEV A-D) and including 120 serotypes. EVs are common human pathogens, transmitted through fecal-oral and respiratory routes. Although the majority of EV infections remains asymptomatic (90 %), these viruses are considered as one of the most common causes of acute viral illnesses in immunocompetent pediatric and adult subjects. High levels of genetic diversity allow these viruses to infect various target cells resulting in a wide spectrum of human acute pathologies including meningitis, respiratory syndromes, cutaneous syndromes, myocarditis and mother-to-child infections. During the early phases of the acute viral infection, EV can modulate the non-specific antiviral strategies developed by the infected target cell (modulation of class I MHC viral antigen presentation ; inhibition of type I interferon expression genes) and to disturb dendritic cell functions resulting in a viral immune escape. This immunological escape allows the generation of genetically modified viruses resulting from RNA genomic deletions, mutations or recombination mechanisms. Persistent replication activities of these genetically modified viruses can induce modulation of specific functions and endocellular pathways of infected cells and the development of chronic inflammatory or autoimmune mechanisms (auto-reactive T and -B cells and auto-antibodies). The persistence of these genetically modified viruses can result in direct or indirect tissue injuries that can explain a subset of chronic myocarditis and dilated cardiomyopathy (DCM), type 1 diabetes mellitus and post-polio syndrome (PPS) cases. Actually no specific and curative therapies are available against EV-induced chronic human pathologies. A better understanding of the molecular mechanisms implicated in viral persistence will stimulate the research into new therapeutic strategies to prevent and treat chronic infections caused by EVs.
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