Emerging biologics for the treatment of pulmonary arterial hypertension

Hye, Tanvirul; Hossain, Md Riajul; Saha, Dipongkor; Foyez, Tahmina; Ahsan, Fakhrul

doi:10.1080/1061186x.2023.2199351

Cited by 5 publications

(3 citation statements)

References 112 publications

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“…To counter the increased endothelin 1 levels, the medical community has been using bosentan, macitentan, and ambrisentan (FDA-approved endothelin receptor antagonists (ERAs) [98]) that inhibit endothelin receptors. Two novel biological treatments that specifically target ETA receptors are being studied: getagozumab, an antibody, and ETR-002 peptide, a treatment that resembles a vaccination [169].…”

Section: Endothelial Function and Pulmonary Therapymentioning

confidence: 99%

Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside

Correale,

Chirivì,

Bevere

et al. 2024

JCM

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Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem to contribute to its onset. To further understand the complex relationship between endothelial and pulmonary hypertension and try to contribute to the development of future therapies, we report a comprehensive and updated review on endothelial function in pulmonary arterial hypertension.

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Section: Endothelial Function and Pulmonary Therapymentioning

confidence: 99%

Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside

Correale,

Chirivì,

Bevere

et al. 2024

JCM

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“…The incidence and prevalence of PAH are estimated at up to 2.4 cases and 15 cases per million annually, respectively 2,3 . Abnormally high mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) are consequences of pathological alterations in various proliferative and inflammatory signaling pathways of pulmonary vascular cells 4,5 . Currently, PAH medication primarily targets the vasoploriferative and vasoconstrictive pathways in pulmonary vasculature.…”

Section: Introductionmentioning

confidence: 99%

“… 2 , 3 Abnormally high mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) are consequences of pathological alterations in various proliferative and inflammatory signaling pathways of pulmonary vascular cells. 4 , 5 Currently, PAH medication primarily targets the vasoploriferative and vasoconstrictive pathways in pulmonary vasculature. Conventional drugs include prostacyclin analogs, endothelin receptor antagonists (ERAs), phosphodiesterase 5 inhibitors (PDE5is), and soluble guanylate cyclase stimulators.…”

Section: Introductionmentioning

confidence: 99%

Impact of different sequential triple oral combination therapies based selexipag on outcomes in pulmonary arterial hypertension

Hu,

Yuan,

Chen

et al. 2024

Clinical Cardiology

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BackgroundWhile the GRIPHON study and others have confirmed the efficacy and safety of selexipag with single, dual, and initial triple combination therapy for patients with pulmonary arterial hypertension (PAH), multicenters studies concerning diverse triple oral combination therapies based on selexipag are limited.HypothesisThis study was conducted to evaluate the effects of various sequential triple oral combination therapies on PAH outcomes.MethodsA retrospective study was carried out involving 192 patients from 10 centers, who were receiving sequential triple oral combination therapy consisting of an endothelin receptor antagonist (ERA), a phosphodiesterase 5 inhibitor (PDE5i)/riociguat and selexipag. Clinical parameters, event‐free survival, and all‐cause survival were assessed and analyzed at baseline and posttreatment.ResultsAmong the 192 patients, 37 were treated with ERA + riociguat + selexipag, and 155 patients received ERA + PDE5i + selexipag. Both sequential triple oral combination therapies improved the World Health Organization functional class and raised the count of low‐risk parameters. As a result of the larger patients' population in the ERA + PDE5i + selexipag group, these individuals exhibited significant increases in 6‐minute walking distance (6MWD), pulmonary arterial systolic pressure, pulmonary arterial pressure, right ventricle, and eccentricity index, and significant decreases in N‐terminal probrain natriuretic peptide after 6 months of treatment. Nevertheless, both sequential triple oral combination therapy groups demonstrated similar shifts in these clinical parameters between baseline and 6 months. Baseline 6MWD and mean pulmonary arterial pressure were independent predictors of survival in patients undergoing ERA + PDE5i + selexipag therapy. Importantly, no significant differences were found in 6‐month event‐free survival and all‐cause survival between two groups.ConclusionsDifferent oral sequential triple combination therapies based on selexipag could comparably improve outcomes in patients with PAH.

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