Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches—2nd Edition

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: 31 October 2026 | Viewed by 1062

Editor

Department of Pharmacology and Toxicology, School of Medicine, University Complutense of Madrid, 28040 Madrid, Spain
Interests: heart failure; cardiovascular system; hypertension; hypertrophy; cardiac function; cardiovascular physiology; cardiomyopathies; echocardiography
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Special Issue Information

Dear Colleagues,

Pulmonary arterial hypertension (PAH) continues to be a condition associated with high levels of morbidity and mortality. The currently available treatments for PAH were developed to restore an imbalance of vasoactive factors. These traditional medications include prostacyclin analogs and receptor agonists, phosphodiesterase 5 inhibitors, endothelin receptor antagonists, and cGMP activators.

However, the progress observed in the medical therapy of PAH patients over the past 15 years is not related to the discovery of new pathways, but to the evolution and testing of new drugs and strategies for combination therapy, and the escalation of treatments based on the systematic assessment of clinical response. These medications prolong life, but mortality remains unacceptably high. Although PAH is a disorder of pulmonary vasculature, right ventricle function is the main prognostic marker and should therefore also be the focus of more detailed analysis for the development of new drugs.

This Special Issue will pay attention to the recent advances in PAH research. We encourage investigators to submit original (basic and translational) research articles and reviews to this Special Issue with the purpose of providing mechanistic insights and deciphering novel diagnostic tools and new treatments for PAH.

Topics include, but are not limited to, the following areas:

  • Cellular, molecular and/or hemodynamic mechanisms that drive PAH;
  • The role of epigenetics in the pathogenesis of PAH;
  • Potential new treatments for PAH;
  • Repurposing established drugs for the resolution of PAH;
  • New perspectives on direct therapies for right heart failure in PAH.

Dr. Rui Adão
Guest Editor

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Keywords

  • pulmonary arterial hypertension
  • right ventricle
  • right heart failure
  • therapy
  • vascular disease
  • translational research

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Research

23 pages, 3436 KB  
Article
From Airways to Arteries: Dissecting the Inflammatory Mechanisms of Pulmonary Vascular Remodeling in a Murine Model of Chronic Airway Inflammation
by Silvia Siragusa, Elena Tantillo, Silvia Parolo, Gloria Modafferi, Maria Laura Faietti, Giulia Natali, Paola Caruso, Sofia Beghi, Silvia Cantoni, Mary Delli Carpini, Maria Giulia Gualandri, Antonella Maria Nogara, Costanza Anna Maria Lagrasta, Vanessa Pitozzi, Maurizio Civelli, Gino Villetti, Enrico Domenici, Marcello Trevisani, Barbara Pioselli and Silvia Pontis
Biomedicines 2026, 14(6), 1359; https://doi.org/10.3390/biomedicines14061359 - 17 Jun 2026
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Abstract
Background: Chronic Obstructive Pulmonary Disease (COPD) is a progressive, incurable condition marked by irreversible airflow limitation and systemic inflammation. Cardiovascular comorbidities, particularly pulmonary hypertension (PH), exacerbate disease severity. While cigarette smoke is a well-known trigger, non-smoking-related inflammatory pathways remain underexplored. This study [...] Read more.
Background: Chronic Obstructive Pulmonary Disease (COPD) is a progressive, incurable condition marked by irreversible airflow limitation and systemic inflammation. Cardiovascular comorbidities, particularly pulmonary hypertension (PH), exacerbate disease severity. While cigarette smoke is a well-known trigger, non-smoking-related inflammatory pathways remain underexplored. This study investigates vascular remodeling in a murine model of inflammation induced by chronic exposure to house dust mite Farinae (HDM). Methods: Female C57BL/6 mice were sensitized with HDM in Freund’s Complete Adjuvant and challenged intranasally with HDM for six weeks. Lung inflammation, mucus hypersecretion, and vascular remodeling were evaluated via BAL, histology, immunofluorescence, echocardiography, gene expression, proteomics, and FlexiVent pulmonary function tests (FlexiVent system). Results: HDM exposure induced a mixed inflammatory response, with elevated neutrophils, monocytes, and lymphocytes in BALF. Mucus hyperproduction (increase in MUC5AC/MUC5B) and impaired lung function (reduced FEV0.1/FVC) were observed. Vascular remodeling was evidenced by increased wall thickness, α-SMA expression, and collagen deposition. Proteomic analysis revealed dysregulation of endothelial markers and protease/antiprotease imbalance. HIF1-α was significantly upregulated in lung tissue and correlated with vascular and epithelial remodeling. Conclusions: Chronic HDM exposure in mice recapitulates key features observed in subsets of COPD and PH, including inflammation-driven airway and vascular remodeling. HIF1-α emerges as a central regulator, linking hypoxia to structural changes. This model offers insights into the effect of non-smoking-related inflammatory pathways on bronchial and vascular remodeling that are potentially relevant for subgroups of COPD patients and highlights HIF1-α as a potential therapeutic target. Full article
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