PulmoCor: National registry for pulmonary hypertension

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The Prognostic Value of Soluble ST2 in Adults with Pulmonary Hypertension

Soluble ST2 (sST2) is upregulated in response to myocardial stress and may serve as biomarker in adults with pulmonary hypertension (PH). This prospective cohort study investigated sST2 levels and its association with echocardiographic and hemodynamic measures, and adverse clinical outcomes in adults with PH of different etiologies. sST2 was measured during the diagnostic right heart catheterization for PH, in adult patients enrolled between May 2012 and October 2016. PH due to left heart failure was excluded. The association between sST2 and a primary endpoint composed of death or lung transplantation and a secondary composite endpoint including death, lung transplantation or heart failure, was investigated using Cox regression with adjustment for NT-proBNP. In total 104 patients were included (median age was 59 years, 66% woman, 51% pulmonary arterial hypertension). Median sST2 was 28 [IQR 20–46] ng/mL. Higher sST2 was associated with worse right ventricular dysfunction and higher mean pulmonary and right atrial pressures. Median follow-up was 3.3 [IQR 2.3–4.6] years. The primary and secondary endpoint occurred in 33 (31.7%) and 43 (41.3%) patients, respectively. sST2 was significantly associated with both endpoints (HR per 2-fold higher value 1.53, 95%CI 1.12–2.07, p = 0.007 and 1.45, 95%CI 1.10–1.90, p = 0.008, respectively). However, after adjustment for NT-proBNP, both associations did not reach statistical significance. In conclusions, higher sST2 levels are associated with more severe PH and right ventricular dysfunction and yields prognostic value in adults with PH, although not independently of NT-proBNP

Correction to: Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Netherlands

Unfortunately the original version of this article contained Electronic Supplementary Material which should not have been published with the article due to copyright reasons. The original version has been updated and the ESM has been removed

Fibrocytes are increased in lung and peripheral blood of patients with idiopathic pulmonary fibrosis

Background: Fibrocytes are implicated in Idiopathic Pulmonary Fibrosis (IPF) pathogenesis and increased proportions in the circulation are associated with poor prognosis. Upon tissue injury, fibrocytes migrate to the affected organ. In IPF patients, circulating fibrocytes are increased especially during exacerbations, however fibrocytes in the lungs have not been examined. Therefore, we sought to evaluate if fibrocytes can be detected in IPF lungs and we compare percentages and phenotypic characteristics of lung fibrocytes with circulating fibrocytes in IPF. Methods: First we optimized flow cytometric detection circulating fibrocytes using a unique combination of intra- and extra-cellular markers to establish a solid gating strategy. Next we analyzed lung fibrocytes in single cell suspensions of explanted IPF and control lungs and compared characteristics and numbers with circulating fibrocytes of IPF. Results: Using a gating strategy for both circulating and lung fibrocytes, which excludes potentially contaminating cell populations (e.g. neutrophils and different leukocyte subsets), we show that patients with IPF have increased proportions of fibrocytes, not only in the circulation, but also in explanted end-stage IPF lungs. These lung fibrocytes have increased surface expression of HLA-DR, increased intracellular collagen-1 expression, and also altered forward and side scatter characteristics compared with their circulating counterparts. Conclusions: These findings demonstrate that lung fibrocytes in IPF patients can be quantified and characterized by flow cytometry. Lung fibrocytes have different characteristics than circulating fibrocytes and represent an intermediate cell population between circulating fibrocytes and lung fibroblast. Therefore, more insight in their phenotype might lead to specific therapeutic targeting in fibrotic lung diseases

Central role of dendritic cells in pulmonary arterial hypertension in human and mice

The pathogenesis of idiopathic pulmonary arterial hypertension (IPAH) is not fully under-stood, but evidence is accumulating tha

Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase

Incidencia del sistema de detracciones en la situación económica y financiera de la empresa de transportes HW E.I.R.L. de la ciudad de Juliaca, periodo 2014 - 2015

TesisEl presente trabajo de investigación titulado “INCIDENCIA DEL SISTEMA DE DETRACCIONES EN LA SITUACIÓN ECONÓMICA Y FINANCIERA DE LA EMPRESA DE TRANSPORTES HW E.I.R.L. DE LA CIUDAD DE JULIACA PERIODO 2014-2015”, se desarrolló con la finalidad de analizar y evaluar la aplicación del Sistema de Detracciones utilizando como instrumento de información a la empresa de Transportes HW E.I.R.L. durante los periodos 2014 – 2015, los resultados han sido obtenidos gracias a los datos recolectados del análisis de los documentos contables, realización de cuadros y gráficos para el debido procesamiento de datos. Las fuentes de información del presente trabajo de investigación fueron el Estado de Situación Financiera, El Estado de Resultados, los libros auxiliares de la empresa en estudio. En la recopilación de información a la empresa de Transportes HW E.I.R.L.se encontró que el Sistema de Detracciones a pesar de que el porcentaje de detracción es minina (4%) ocasiona una disminución en la liquidez, el nivel de liquidez de la empresa en estudio en los periodos 2014 – 2015, fueron de 1.76 y 1.21 para cubrir cada nuevo sol del pasivo corriente, la razón acida muestra una disminución en la capacidad de pago de 1.24 y 0.97 para pagar las deudas a corto plazo y finalmente al evaluar la liquidez absoluta disminuyo a 0.77 y 0.76. En relación al capital de trabajo se obtuvo una disminución con la aplicación del sistema de detracciones a 50,378.64 y 21,876.36 en los dos últimos periodos. Y como segundo análisis realizado a la empresa se tomó el indicador de rentabilidad económica, ya que se observó dinero inmovilizado en la cuenta de detracciones del Banco de la Nación ya que los montos detraídos ascienden a 11,346.39 y 11,728.10 los mismos que representan el 2.98% y 2.92% del total de los activos, la realidad de la empresa respecto a su rentabilidad es de 3.14% y 5.22% para los periodos 2014 y 2015, finalmente para el tercer análisis se observó que las detracciones cubren el total de los tributos a pagar e inclusive existe un saldo para el siguiente pago de tributos para el año 2014 tuvo un saldo de 2,012.27 y para el 2015 se incrementó a 2956.5

Plasma markers in pulmonary hypertension subgroups correlate with patient survival

Background: Recent studies have provided evidence for an important contribution of the immune system in the pathophysiology of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). In this report, we investigated whether the inflammatory profile of pulmonary hypertension patients changes over time and correlates with patient WHO subgroups or survival. Methods: 50 PAH patients (16 idiopathic (I)PAH, 24 Connective Tissue Disease (CTD)-PAH and 10 Congenital Heart Disease (CHD)-PAH), 37 CTEPH patients and 18 healthy controls (HCs) were included in the study. Plasma inflammatory markers at baseline and after 1-year follow-up were measured using ELISAs. Subsequently, correlations with hemodynamic parameters and survival were explored and data sets were subjected to unbiased multivariate analyses. Results: At diagnosis, we found that plasma levels of interleukin-6 (IL-6) and the chemokines (C-X3-C) motif legend CXCL9 and CXCL13 in CTD-PAH patients were significantly increased, compared with HCs. In idiopathic PAH patients the levels of tumor growth factor-β (TGFβ), IL-10 and CXCL9 were elevated, compared with HCs. The increased CXCL9 and IL-8 concentrations in CETPH patients correlated significantly with decreased survival, suggesting that CXCL9 and IL-8 may be prognostic markers. After one year of treatment, IL-10, CXCL13 and TGFβ levels changed significantly in the PAH subgroups and CTEPH patients. Unbiased multivariate analysis revealed clustering of PH patients based on inflammatory mediators and clinical parameters, but did not separate the WHO subgroups. Importantly, these multivariate analyses separated patients with &lt; 3 years and &gt; 3 years survival, in particular when inflammatory mediators were combined with clinical parameters. Discussion: Our study revealed elevated plasma levels of inflammatory mediators in different PAH subgroups and CTEPH at baseline and at 1-year follow-up, whereby CXCL9 and IL-8 may prove to be prognostic markers for CTEPH patients. While this study is exploratory and hypothesis generating, our data indicate an important role for IL-8 and CXCL9 in CHD and CTEPH patients considering the increased plasma levels and the observed correlation with survival. Conclusion: In conclusion, our studies identified an inflammatory signature that clustered PH patients into WHO classification-independent subgroups that correlated with patient survival.</p