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2.
Ann Hepatol ; 2020 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-32418749

RESUMO

INTRODUCTION & OBJECTIVES: Liver cirrhosis is a major cause of mortality worldwide. Adequate diagnosis and treatment of decompensating events requires of both medical skills and updated technical resources. The objectives of this study were to search the demographic profile of hospitalized cirrhotic patients in a group of Latin American hospitals and the availability of expertise/facilities for the diagnosis and therapy of decompensation episodes. METHODS: A cross sectional, multicenter survey of hospitalized cirrhotic patients. RESULTS: 377 patients, (62% males; 58±11 years) (BMI>25, 57%; diabetes 32%) were hospitalized at 65 centers (63 urbans; 57 academically affiliated) in 13 countries on the survey date. Main admission causes were ascites, gastrointestinal bleeding, hepatic encephalopathy and spontaneous bacterial peritonitis/other infections. Most prevalent etiologies were alcohol-related (AR) (40%); non-alcoholic-steatohepatitis (NASH) (23%), hepatitis C virus infection (HCV) (7%) and autoimmune hepatitis (AIH) (6%). The most frequent concurrent etiologies were AR+NASH. Expertise and resources in every analyzed issue were highly available among participating centers, mostly accomplishing valid guidelines. However, availability of these facilities was significantly higher at institutions located in areas with population>500,000 (n=45) and in those having a higher complexity level (Gastrointestinal, Liver and Internal Medicine Departments at the same hospital (n=22). CONCLUSIONS: The epidemiological etiologic profile in hospitalized, decompensated cirrhotic patients in Latin America is similar to main contemporary emergent agents worldwide. Medical and technical resources are highly available, mostly at great population urban areas and high complexity medical centers. Main diagnostic and therapeutic approaches accomplish current guidelines recommendations.

3.
Arch Bronconeumol ; 2020 May 18.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-32439253

RESUMO

BACKGROUND: There is uncertainty regarding efficacy of telehealth-based approaches in COPD patients for sustaining benefits achieved with intensive pulmonary rehabilitation (PR). RESEARCH QUESTION: To determine whether a maintenance pulmonary telerehabilitation (TelePR) programme, after intensive initial PR, is superior to usual care in sustaining over time benefits achieved by intensive PR. STUDY DESIGN AND METHODS: A multicentre open-label pragmatic parallel-group randomized clinical trial was conducted. Two groups were created at completion of an 8-week intensive outpatient hospital PR programme. Intervention group (IG) patients were given appropriate training equipment and instructed to perform three weekly training sessions and send performance data through an app to a web-based platform. Patients in the control group (CG) were advised to exercise regularly (usual care). RESULTS: Ninety-four patients (46 IG, 48 CG) were randomized. The analysis of covariance showed non-significant improvements in 6-min walk distance [19.9m (95% CI -4.1/+43.8)] and Chronic Respiratory Disease Questionnaire - Emotion score [0.4 points (0-0.8)] in the IG. Secondary linear mixed models showed improvements in the IG in Short Form-36 mental component summary [9.7, (4.0-15.4)] and Chronic Respiratory Disease Questionnaire - Emotion [0.5, (0.2-0.9)] scores, but there was no association between compliance and outcomes. Acute exacerbations were associated with a marginally significant decrease in 6-minute walk distance of 15.8m (-32.3/0.8) in linear models. CONCLUSIONS: The TelePR maintenance strategy was both feasible and safe but failed to show superiority over usual care, despite improvements in some HRQoL domains. Acute exacerbations may have an important negative influence on long-term physical function. CLINICALTRIALS. GOV IDENTIFIER: NCT03247933.

4.
Immunity ; 2020 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-32428502

RESUMO

Cell death pathways regulate various homeostatic processes. Autoimmune lymphoproliferative syndrome (ALPS) in humans and lymphoproliferative (LPR) disease in mice result from abrogated CD95-induced apoptosis. Because caspase-8 mediates CD95 signaling, we applied genetic approaches to dissect the roles of caspase-8 in cell death and inflammation. Here, we describe oligomerization-deficient Caspase-8F122GL123G/F122GL123G and non-cleavable Caspase-8D387A/D387A mutant mice with defective caspase-8-mediated apoptosis. Although neither mouse developed LPR disease, removal of the necroptosis effector Mlkl from Caspase-8D387A/D387A mice revealed an inflammatory role of caspase-8. Ablation of one allele of Fasl, Fadd, or Ripk1 prevented the pathology of Casp8D387A/D387AMlkl-/- animals. Removing both Fadd alleles from these mice resulted in early lethality prior to post-natal day 15 (P15), which was prevented by co-ablation of either Ripk1 or Caspase-1. Our results suggest an in vivo role of the inflammatory RIPK1-caspase-8-FADD (FADDosome) complex and reveal a FADD-independent inflammatory role of caspase-8 that involves activation of an inflammasome.

5.
Environ Pollut ; 263(Pt A): 114480, 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-32283460

RESUMO

Bioaccumulation of Hg and Cd from food is a complex ecological process that has been oversimplified in the past. Common dolphins (Delphinus delphis) provide a powerful model to biomonitor metal concentrations in marine environments worldwide. We combined proportions-based nutritional geometry with metal analysis, stomach content analysis and the proximate composition of prey, to yield novel insights into the accumulation of Hg and Cd. Our analysis showed an age-related accumulation trend for Cd and Hg in kidney and liver, with highest concentrations found at 18 years of age. When viewed through the lens of nutritional ecology, Argentine anchovy (58.1 Mass %) and South American long-finned squid (22.7 Mass %), provided most of the dietary intake of protein (P) and lipids (L) (P:L ratio = 2.6:1.0) and also represented the main source for Cd and Hg levels accumulated in their bodies. This study presents unprecedented evidence on metal accumulation in relation to age and nutritional intake in a marine predator.

6.
Phys Rev Lett ; 124(13): 136402, 2020 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-32302162

RESUMO

Employing high-pressure infrared spectroscopy we unveil the Weyl semimetal phase of elemental Te and its topological properties. The linear frequency dependence of the optical conductivity provides clear evidence for metallization of trigonal tellurium (Te-I) and the linear band dispersion above 3.0 GPa. This semimetallic Weyl phase can be tuned by increasing pressure further: a kink separates two linear regimes in the optical conductivity (at 3.7 GPa), a signature proposed for Type-II Weyl semimetals with tilted cones; this however reveals a different origin in trigonal tellurium. Our density-functional calculations do not reveal any significant tilting and suggest that Te-I remains in the Type-I Weyl phase, but with two valence bands in the vicinity of the Fermi level. Their interplay gives rise to the peculiar optical conductivity behavior with more than one linear regime. Pressure above 4.3 GPa stabilizes the more complex Te-II and Te-III polymorphs, which are robust metals.

7.
Artigo em Inglês | MEDLINE | ID: mdl-32297099

RESUMO

To investigate the value of tissue Doppler velocities for ruling out treatment-requiring acute cellular rejection (TR-ACR), in the context of myocardial deformation analysis performed by means of speckle tracking echocardiography. We performed serial echocardiograms in 37 heart transplant recipients in their first year post-transplantation within 3 h of the routine surveillance endomyocardial biopsies (EMB). The association of the sum of lateral mitral annulus systolic (s') and early diastolic (e') velocities, in absolute values, measured by tissue Doppler echocardiography (s'+ e'), with TR-ACR (ACR grade ≥ 2R) was investigated by multivariate analysis, including classic echocardiographic parameters and myocardial deformation variables. A total of 251 pairs of EMB and echo exams were performed, 35 (14%) with rejection grade ≥ 2R (TR-ACR). s' + e' was independently associated to TR-ACR (OR 0.80, 95%CI 0.72-0.89, p < 0.0005), with a C statistic of 0.79 (95%CI 0.71-0.87, p < 0.0005) by ROC curve analysis. An s'+ e' value ≥ 23 cm/s, present in 43% of studies, had a negative predictive value of 98% for ruling out TR-ACR. Moreover, in the same patients, s'+ e' significantly decreased when TR-ACR occurred after a study without this condition (- 3.7 ± 3.3 cm/s, p = 0.003), but it was similar when rejection status was the same in the present versus the previous study. A drop in s'+ e' value < 2.7 cm/s from the previous echocardiogram, had a 99% negative predictive value for ruling out TR-ACR. Tissue Doppler velocities, a widely available echo parameter, were found to be a valuable marker for ruling out TR-ACR in this multivariate study which included myocardial deformation variables.

9.
Rev. méd. Urug ; 36(1): 153-191, mar. 2020. tab, graf
Artigo em Espanhol | LILACS-Express | ID: biblio-1094227

RESUMO

Resumen: Introducción: los cigarrillos electrónicos (CE) son sistemas que liberan aerosol conteniendo nicotina y otras sustancias. Desde su aparición han generado una gran controversia en la comunidad científica dedicada al control de tabaco, existiendo evidencias que los posicionan como una alternativa de tratamiento para la cesación tabáquica y otros estudios que muestran sus daños a la salud, así como convertirlos en la puerta de entrada a los jóvenes a la adicción a nicotina. Objetivo: recopilar evidencia disponible sobre CE con referencia a componentes, riesgos para la salud, cesación de tabaquismo, impacto poblacional y aspectos normativos y regulatorios. Método: revisión narrativa. Se realizó búsqueda bibliográfica en bases PubMed, MEDLINE, SCOPUS, Redalyc, SciELO, LiLACS, Dialnet, ScienceDirect, así como en documentos de la Organización Mundial de la Salud, sociedades científicas, sitios web dedicados a consumidores y legislación nacional e internacional. Resultados: se revisaron 130 documentos, de los cuales se toman los 63 más relevantes. Existen evidencias científicas sobre contenidos tóxicos y carcinógenos en líquidos y aerosoles de los CE, así como de daño a la salud cardiovascular y respiratoria. Se presentan estudios controversiales sobre su eficacia para dejar de fumar. Se presenta evidencia acerca de cómo los CE impactan negativamente en la iniciación de jóvenes al consumo y en la recaída de exfumadores. Se analiza la normativa nacional e internacional para regular los CE. Conclusiones: los profesionales de la salud no deben aconsejar el uso de CE, dado que no son seguros y exponen a los consumidores y a la población en general de los efectos deletéreos directos e indirectos de los CE.


Summary: Introduction: e-cigarettes are systems that produce and release aerosol that contains nicotine and other substances. They have been controversial in the scientific community that works on tobacco control ever since they were created. There is evidence suggesting they are an alternative therapy for tobacco cessation and other studies prove they are harmful to health, and they end up being the door that welcomes young people to the world of nicotine addiction. Objective: to collect evidence on e-cigarette in connection with: components, risks to the health, tobacco cessation, impact on the population and normative and regulatory aspects. Method: Review of the literature. Bibliographic search on PubMed, MEDLINE, SCOPUS, Redalyc, Scielo, LiLACS, Dialnet, ScienceDirect, as well as in other documents of the World Health Organization, scientific societies, websites dedicated to consumers and the national and international laws. Results: 130 documents were reviewed, and the 63 most relevant ones served as the grounds for the study. There is scientific evidence on the toxic and carcinogenic content of liquids and aerosols of e-cigarettes, as well as on their negative effect on cardiovascular and respiratory health. We present controversial studies on its effectiveness to give up smoking, evidence on how e-cigarettes have a negative influence on the initiation of consumption by the young and on ex-smokers relapse. The study also analyses the national and international norms regulating e-cigarettes. Conclusions: health professionals must not recommend the use of e-cigarettes, since they are not safe and expose consumers and the general population to its direct and indirect harmful effects.


Resumo: Introdução: os cigarros eletrônicos (CE) são sistemas que liberam aerossol contendo nicotina e outras substâncias. Desde seu surgimento geraram uma grande controvérsia na comunidade científica dedicada ao controle do tabaco, existindo evidências que os colocam como uma alternativa de tratamento para a cessação do tabagismo e outros estudos que mostram seus danos à saúde, e como se transformam na porta de entrada dos jovens a adição a nicotina. Objetivo: recuperar a evidência disponível sobre CE com referência a: componentes, riscos para a saúde, cessação de tabagismo, impacto populacional e aspectos normativos e regulamentares. Método: revisão narrativa. Fez-se uma pesquisa bibliográfica nas bases PubMed, MEDLINE, SCOPUS, Redalyc, Scielo, LiLACS, Dialnet, ScienceDirect, e também em publicações da Organização Mundial da Saúde, sociedades científicas, sites web dedicados a consumidores e legislação nacional e internacional. Resultados: foram selecionados os 63 documentos mais relevantes dos 130 recuperados. Existem evidências científicas sobre conteúdos tóxicos e carcinogênicos em líquidos e aerossóis dos CE e também dos danos à saúde cardiovascular e respiratória. Apresentam-se estudos controversos sobre sua eficácia para apoiar o abandono do hábito de fumar e evidência de como os CE têm um impacto negativo na iniciação de jovens ao consumo e na recaída de ex- fumantes. Faz-se uma análise das normas nacionais e internacionais para regular os CE. Conclusões: os profissionais de saúde não devem aconselhar o uso de CE por não ser seguros e por que expõem os consumidores e a população em geral aos efeitos deletérios diretos e indiretos de seu uso.

10.
Cell ; 180(6): 1115-1129.e13, 2020 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-32200799

RESUMO

Influenza A virus (IAV) is a lytic RNA virus that triggers receptor-interacting serine/threonine-protein kinase 3 (RIPK3)-mediated pathways of apoptosis and mixed lineage kinase domain-like pseudokinase (MLKL)-dependent necroptosis in infected cells. ZBP1 initiates RIPK3-driven cell death by sensing IAV RNA and activating RIPK3. Here, we show that replicating IAV generates Z-RNAs, which activate ZBP1 in the nucleus of infected cells. ZBP1 then initiates RIPK3-mediated MLKL activation in the nucleus, resulting in nuclear envelope disruption, leakage of DNA into the cytosol, and eventual necroptosis. Cell death induced by nuclear MLKL was a potent activator of neutrophils, a cell type known to drive inflammatory pathology in virulent IAV disease. Consequently, MLKL-deficient mice manifest reduced nuclear disruption of lung epithelia, decreased neutrophil recruitment into infected lungs, and increased survival following a lethal dose of IAV. These results implicate Z-RNA as a new pathogen-associated molecular pattern and describe a ZBP1-initiated nucleus-to-plasma membrane "inside-out" death pathway with potentially pathogenic consequences in severe cases of influenza.

11.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-32205100

RESUMO

INTRODUCTION AND OBJECTIVES: Two-dimensional speckle-tracking echocardiography has emerged as a promising alternative to endomyocardial biopsy to rule out acute cellular rejection after orthotopic heart transplantation (OHT) in single center studies. In an original cohort, 15.5% and 17% of cutoff points for left ventricular global longitudinal strain (LVGLS) and free-wall right ventricular longitudinal strain, respectively, achieved 100% negative predictive value to exclude moderate or severe acute cellular rejection (ACR ≥ 2R). Our objective was to demonstrate the usefulness of speckle-tracking and validate these cutoff points in an external cohort. METHODS: A prospective, multicenter study that included patients who were monitored during their first year after OHT was conducted. Echocardiographic studies analyzed by local investigators were compared with simultaneous paired endomyocardial biopsies samples. RESULTS: A total of 501 endomyocardial biopsy-echocardiographic studies were included in 99 patients. ACR≥2R was present in 7.4% of samples. LVGLS and free-wall right ventricular longitudinal strain were significantly reduced during ACR≥2R on univariate analysis. On multivariate analysis, LVGLS was independently associated with the presence of ACR≥2R. The original cutoff points demonstrated a negative predictive value of 94.3% to exclude ACR≥2R. CONCLUSIONS: This study maintained a strong negative predictive value to exclude ACR≥2R after OHT and LVGLS was independently associated with the presence of ACR≥2R. We propose the use of speckle-tracking, especially LVGLS, as part of the noninvasive diagnosis and management of ACR.

13.
Soft Matter ; 16(5): 1162-1169, 2020 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-31913382

RESUMO

In this work we have characterized the phase behaviour and the dynamics of bidimensional mixtures of active and passive Brownian particles. We have evaluated state diagrams at several concentrations of the passive components finding that, while passive agents tend to hinder phase separation, active agents force crystal-like structures on passive colloids. In order to study how passive particles affect the dynamics of the mixture, we have computed the long-time diffusion coefficient of each species, concluding that active particles induce activity and super-diffusive behaviour on passive ones. Interestingly, at the density at which the system enters a MIPS state the active particles' diffusivity shows an inflection point and the passive particles' one goes through a maximum, due to the change in the dynamics of the active components, as shown in the displacement's probability distribution function.

14.
Sci Total Environ ; 712: 135518, 2020 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-31806303

RESUMO

Coastal areas are vulnerable and fluctuating habitats that include highly valuable spaces for habitat and species conservation and, at the same time, they are among the most invaded ecosystems worldwide. Occupying large areas within Mediterranean-climate coastlines, the "ecosystem engineer" Carpobrotus edulis appears as a menace for coastal biodiversity and ecosystem services. By combining the observation, current distribution, glasshouse experiment, and dispersion modeling, we aim to achieve a better understanding of the successful invasion process and potential dispersion patterns of C. edulis. We analyzed the response of plant propagules (seeds and plant fragments) to seawater immersion during increasing periods of time (up to 144 h). After 2 months of growth, plant fragments showed a total survival rate (100%) indicating high tolerance to salinity. During this time, fragment length was increased (up to 60%) and root length was higher than control in all cases. Also, immersed fragments consistently accumulated more biomass than control fragments. After two months of growth, photosynthetic parameters (Fv'/Fm', ΦNO, and ΦII) remained stable compared to control fragments. Physiologically, osmolyte and pigment content did not evidence significant changes regardless of immersion time. Based on the capacity of propagules to survive seawater immersion, we modeled the potential transport of C. edulis by combining an oceanic model (ROMS-AGRIF) with a particle-tracking model. Results indicated that propagules may travel variable distances maintaining physiological viability. Our model suggested that short-scale circulation would be the dominant process, however, long-scale circulation of propagules may be successfully accomplished in <6 days. Furthermore, under optimal conditions (southerly winds dominance), propagules may even travel large distances (250 km alongshore). Modeling transport processes, in combination with the dynamics of introduction and expansion, will contribute to a better understanding of the invasive mechanisms of C. edulis and, consequently, to design preventive strategies to reduce the impact of plant invasion.

15.
J Endocrinol ; 244(1): 83-94, 2020 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-31585438

RESUMO

Hypothyroidism is often associated with anemia and immunological disorders. Similar defects are found in patients and in mice with a mutated dominant-negative thyroid hormone receptor α (TRα) and in knockout mice devoid of this receptor, suggesting that this isoform is responsible for the effects of the thyroid hormones in hematopoiesis. However, the hematological phenotype of mice lacking also TRß has not yet been examined. We show here that TRα1/TRß-knockout female mice, lacking all known thyroid hormone receptors with capacity to bind thyroid hormones, do not have overt anemia and in contrast with hypothyroid mice do not present reduced Gata1 or Hif1 gene expression. Similar to that found in hypothyroidism or TRα deficiency during the juvenile period, the B-cell population is reduced in the spleen and bone marrow of ageing TRα1/TRß-knockout mice, suggesting that TRß does not play a major role in B-cell development. However, splenic hypotrophy is more marked in hypothyroid mice than in TRα1/TRß-knockout mice and the splenic population of T-lymphocytes is not significantly impaired in these mice in contrast with the reduction found in hypothyroidism. Our results show that the overall hematopoietic phenotype of the TRα1/TRß-knockout mice is milder than that found in the absence of hormone. Although other mechanism/s cannot be ruled out, our results suggest that the unoccupied TRs could have a negative effect on hematopoiesis, likely secondary to repression of hematopoietic gene expression.

16.
Arq Bras Cardiol ; 113(5): 960-968, 2019 Nov.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31800721

RESUMO

BACKGROUND: Coronary angiography with two catheters is the traditional strategy for diagnostic coronary procedures. TIG I catheter permits to cannulate both coronary arteries, avoiding exchanging catheters during coronary angiography by transradial access. OBJECTIVE: The aim of this study is to evaluate the impact of one-catheter strategy, by avoiding catheter exchange, on coronary catheterization performance and economic costs. METHODS: Transradial coronary diagnostic procedures conducted from January 2013 to June 2017 were collected. One-catheter strategy (TIG I catheter) and two-catheter strategy (left and right Judkins catheters) were compared. The volume of iodinated contrast administered was the primary endpoint. Secondary endpoints included radial spasm, procedural duration (fluoroscopy time) and exposure to ionizing radiation (dose-area product and air kerma). Direct economic costs were also evaluated. For statistical analyses, two-tailed p-values < 0.05 were considered statistically significant. RESULTS: From a total of 1,953 procedures in 1,829 patients, 252 procedures were assigned to one-catheter strategy and 1,701 procedures to two-catheter strategy. There were no differences in baseline characteristics between the groups. One-catheter strategy required less iodinated contrast [primary endpoint; (60-105)-mL vs. 92 (64-120)-mL; p < 0.001] than the two-catheter strategy. Also, the one-catheter group presented less radial spasm (5.2% vs. 9.3%, p = 0.022) and shorter fluoroscopy time [3.9 (2.2-8.0)-min vs. 4.8 (2.9-8.3)-min, p = 0.001] and saved costs [149 (140-160)-€/procedure vs. 171 (160-183)-€/procedure; p < 0.001]. No differences in dose-area product and air kerma were detected between the groups. CONCLUSIONS: One-catheter strategy, with TIG I catheter, improves coronary catheterization performance and reduces economic costs compared to traditional two-catheter strategy in patients referred for coronary angiography.

17.
Acta Neuropathol Commun ; 7(1): 219, 2019 12 23.
Artigo em Inglês | MEDLINE | ID: mdl-31870437

RESUMO

Synucleinopathies are mostly sporadic neurodegenerative disorders of partly unexplained aetiology, and include Parkinson's disease (PD) and multiple system atrophy (MSA). We have further investigated our recent finding of somatic SNCA (α-synuclein) copy number variants (CNVs, specifically gains) in synucleinopathies, using Fluorescent in-situ Hybridisation for SNCA, and single-cell whole genome sequencing for the first time in a synucleinopathy. In the cingulate cortex, mosaicism levels for SNCA gains were higher in MSA and PD than controls in neurons (> 2% in both diseases), and for MSA also in non-neurons. In MSA substantia nigra (SN), we noted SNCA gains in > 3% of dopaminergic (DA) neurons (identified by neuromelanin) and neuromelanin-negative cells, including olig2-positive oligodendroglia. Cells with CNVs were more likely to have α-synuclein inclusions, in a pattern corresponding to cell categories mostly relevant to the disease: DA neurons in Lewy-body cases, and other cells in the striatonigral degeneration-dominant MSA variant (MSA-SND). Higher mosaicism levels in SN neuromelanin-negative cells may correlate with younger onset in typical MSA-SND, and in cingulate neurons with younger death in PD. Larger sample sizes will, however, be required to confirm these putative findings. We obtained genome-wide somatic CNV profiles from 169 cells from the substantia nigra of two MSA cases, and pons and putamen of one. These showed somatic CNVs in ~ 30% of cells, with clonality and origins in segmental duplications for some. CNVs had distinct profiles based on cell type, with neurons having a mix of gains and losses, and other cells having almost exclusively gains, although control data sets will be required to determine possible disease relevance. We propose that somatic SNCA CNVs may contribute to the aetiology and pathogenesis of synucleinopathies, and that genome-wide somatic CNVs in MSA brain merit further study.

18.
Echocardiography ; 36(12): 2185-2194, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31756023

RESUMO

PURPOSE: Our objective was to investigate the impact of inter-vendor variability in the ability of myocardial strain analysis to detect acute cellular rejection (ACR) in heart transplant recipients. METHODS: We performed serial echocardiographic examinations in 18 consecutive adult heart transplanted patients, in their first year post-transplantation, within 3 hours of the routine surveillance endomyocardial biopsies (EMB) in a single center. Myocardial strain was analyzed using two software in two different institutions, and inter-vendor variability of strain values and its association with ACR (any grade or grade ≥2R) was investigated. The parameter of comparison was the peak value of the average curve of strain during the entire cardiac cycle. RESULTS: A total of 147 pairs of EMB-echocardiogram were performed, 65 with no ACR, 63 with ACR grade 1R, and 19 with ACR grade ≥2R. Intra-class correlation coefficients for left ventricle longitudinal, radial, and circumferential strain were 0.38, 0.39, and 0.77, respectively, and 0.32 for right ventricular longitudinal strain. Neither software found significant association of left ventricular longitudinal strain with rejection. Grade ≥2R ACR was associated with left ventricular circumferential strain measured with the first software and with left ventricular radial strain with the other; and ACR of any grade was only significantly associated with right ventricle longitudinal strain measured with the first software. CONCLUSIONS: Inter-vendor reproducibility of strain values was low in this study. Some strain parameters were associated to ACR, although these results were inconsistent between two commercially available software. Specific validation of each software is warranted for this clinical indication.

20.
Artigo em Inglês | MEDLINE | ID: mdl-31702781

RESUMO

AIMS: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a life-threatening entity with a highly heterogeneous genetic background. Cardiac magnetic resonance (CMR) imaging can identify fibrofatty scar by late gadolinium enhancement (LGE). Our aim is to investigate genotype-phenotype correlation in ARVC/D mutation carriers, focusing on CMR-LGE and myocardial fibrosis patterns. METHODS AND RESULTS: A cohort of 44 genotyped patients, 33 with definite and 11 with borderline ARVC/D diagnosis, was characterized using CMR and divided into groups according to their genetic condition (desmosomal, non-desmosomal mutation, or negative). We collected information on cardiac volumes and function, as well as LGE pattern and extension. In addition, available ventricular myocardium samples from patients with pathogenic gene mutations were histopathologically analysed. Half of the patients were women, with a mean age of 41.6 ± 17.5 years. Next-generation sequencing identified a potential pathogenic mutation in 71.4% of the probands. The phenotype varied according to genetic status, with non-desmosomal male patients showing lower left ventricular (LV) systolic function. LV fibrosis was similar between groups, but distribution in non-desmosomal patients was frequently located at the posterolateral LV wall; a characteristic LV subepicardial circumferential LGE pattern was significantly associated with ARVC/D caused by desmin mutation. Histological analysis showed increased fibrillar connective tissue and intercellular space in all the samples. CONCLUSION: Desmosomal and non-desmosomal mutation carriers showed different morphofunctional features but similar LV LGE presence. DES mutation carriers can be identified by a specific and extensive LV subepicardial circumferential LGE pattern. Further studies should investigate the specificity of LGE in ARVC/D.

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