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Pulmonary Arterial Hypertension - Springer Nature

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Last Updated: 08 August 2022

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Adjusting Overall Survival Estimates of Macitentan in Pulmonary Arterial Hypertension After Treatment Switching: Results from the SERAPHIN Study

SERAPHIN, a large RCT, analyzed the effects of long-term treatment with the endothelin receptor antagonist macitentan in patients with pulmonary arterial hypertension. Methods The inverse probability of censoring weighted and age-preserving structural failure time models were used to determine the effect on overall mortality when there was no treatment change in SERAPHIN. Patients in the placebo group switched to open-label macitentan 10 mg were delayed, and patients in the macitentan 10 mg group were among the few people to stop using macitentan earlier this year. Study Conclusions The placebo group's patients had switched to macitentan 10 mg after the study was ended, and 73. 2% of patients in the placebo group had moved to macitentan 10 mg. Among these patients, exposure to macitentan 10 mg was 28. 2% of total study treatment exposure, according to 28. 2%. The macitentan 10 mg group did not receive open-label macitentan for 44. 8 percent at study's end, and 24. 8% of patients were not receiving macitentan; the average time not receiving macitentan was 44. 3 weeks.

Source link: https://doi.org/10.1007/s12325-022-02253-8


Platelet Activation Markers in Children with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

The study sought to determine mean platelet volume, platelet distribution width, and platecrit in children with congenital heart disease to determine the predictive value of these platelet activation markers for adverse outcomes and compare their results with different studies in these patients. This prospective cohort study included 60 children with PAH-CHD as group I and 60 children with CHD and no PAH as group II. Right ventricular diameter and mean pulmonary arterial pressure were positively related with right ventricular diameter and mean pulmonary artery pressure, but they did not positively correlate with right ventricular systolic and diastolic function. MPV was the best cut-off of platelet activation markers to predict poor prognosis in group I, with 75% sensitivity and 61. 5% specificity for PDW, and 93. 2% specificity for platecrit.

Source link: https://doi.org/10.1007/s00246-022-02847-7


Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension

Objective Connective tissue disease association pulmonary hypertension is classified as a subgroup of WHO group 1 PH, which also called pulmonary arterial hypertension. CTD-PH patients were enrolled and divided into WHO group1 PH, WHO group 2 PH, and hyperdynamic PH > 20 mmHg, PVR. PPC> 20 mmHg, PVR> 20 mmHg, PVR > 20 mmHg.

Source link: https://doi.org/10.1186/s12890-022-02081-0


Kynurenine metabolites predict survival in pulmonary arterial hypertension: A role for IL-6/IL-6Rα

In patients with pulmonary arterial hypertension undergoing PAH therapy, the kynurenine pathway has been activated. We set out to determine KP-metabolism in treatment-nave PAH patients, investigate its prognostic effects, evaluate the effect of PAH therapy on KP-metabolites, and identify cytokines responsible for altered KP-metabolism. At the time of diagnosis, six months and one year after PAH therapy, blood sampling of PAH patients was done. Patients with lower tryptophan, higher kynurenine, 3-hydroxykynurenine, quinolinic acid, kynurenine, azo urenic acid, kynurenine, nurenine, 3-hydroxykynurenine, hydroxykynurenine, quinolinic acid, kynurenine, knurenine, kynurenine, phosphonurenine, te compared to controls had higher tryptophan, kynurenine kynurenine, 3-hydroxykynurenine, hydroxykynurenine,, hydroxykynurenine, kynurenine,,,, hydroxykynurenine,, kynurenine, kyn In vitro, human lung primary cells were exposed to various cytokines. Both cell types have a similar KP-metabolite profile as seen in PAH patients following exposure to the interleukin-6 /IL-6 receptor u03b1 complex. PAH therapy and survival of PAH patients are predicted by KP-metabolites.

Source link: https://doi.org/10.1038/s41598-022-15039-3


Prostaglandin E1 reduces apoptosis and improves the homing of mesenchymal stem cells in pulmonary arterial hypertension by regulating hypoxia-inducible factor 1 alpha

In this research, we preconditioned the MSCs with prostaglandin E1 and carried out in vitro and in vivo cell experiments to determine the therapeutic benefits of MSCs in rats with PAH. Preconditioning with PGE1 raised the protein levels of HIF-1 alpha in MSCs, which can reduce MSC apoptosis and raise the protein levels of CXCR4, MSC migration, and vascular endothelial growth factor secretion. The pulmonary artery systolic pressure, mean pulmonary arterial artery pressure, right ventricular hypertrophy index, %WT, and %WA decreased in rats with PAH following injection with PGE1-PCMSCs. PGE1 modulates MSC characteristics by regulating the HIF pathway, providing insight into the process by which PGE1 preconditioning can be used to increase MSCs' therapeutic ability in PAH.

Source link: https://doi.org/10.1186/s13287-022-03011-x


Long-Term Safety, Tolerability and Survival in Patients with Pulmonary Arterial Hypertension Treated with Macitentan: Results from the SERAPHIN Open-Label Extension

macitentan 10 mg in PAH patients' long-term safety and tolerability are both evaluated by the company's open-label extension study. Methods Patients in SERAPHIN who completed the double-blind treatment period or suffered with a morbidity event during the research may have been eligible for SERAPHIN OL. Patients in SERAPHIN OL were randomly administered macitentan 10 mg in SERAPHIN, and two closely related sets were tested for safety: all patients in SERAPHIN OL; patients in SERAPHIN OL were randomly randomized to macitentan 10 mg in SERAPHIN. In SERAPHIN, 742 patients were randomised in SERAPHIN, 550 became SERAPHIN OL; 242 patients were randomly exposed to macitentan 10 mg;. Macitentan 10 mg's safety profile after this lengthy treatment period was in accordance with that observed in SERAPHIN. Our report, which was included as part of mixed therapy, provides more insight into the long-term stability of macitentan as the majority of patients were receiving other PAH therapy at macitentan's start.

Source link: https://doi.org/10.1007/s12325-022-02199-x

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions