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Biomedicines
Special Issues
Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches
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Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches

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

Deadline for manuscript submissions: 31 May 2025 | Viewed by 2261

Special Issue Editor

Dr. Rui Adão

E-Mail Website
Guest 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

Special Issue Information

Dear Colleagues,

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

However, 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 a 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 a more detailed analysis for new drug development in PAH.

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 to address mechanistic insights and decipher novel diagnostic tools and new treatments for PAH.

Topics include, but are not limited to:

  • 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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

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

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Published Papers (3 papers)

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Research

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25 pages, 6165 KiB  
Article
Pulmonary Arterial Hypertension-Induced Reproductive Damage: Effects of Combined Physical Training on Testicular and Epididymal Parameters in Rats
by Mírian Quintão Assis, Luciano Bernardes Leite, Luiz Otávio Guimarães-Ervilha, Rui Adão, Emily Correna Carlo Reis, Antônio José Natali and Mariana Machado-Neves
Biomedicines 2025, 13(2), 410; https://doi.org/10.3390/biomedicines13020410 - 8 Feb 2025
Viewed by 401
Abstract
Background/Objectives: Pulmonary arterial hypertension (PAH) affects the pulmonary vasculature and cardiac function. While its impact on target organs has been extensively studied, little is known about its effects on highly vascularized organs, such as those from the male reproductive system. This study explores [...] Read more.
Background/Objectives: Pulmonary arterial hypertension (PAH) affects the pulmonary vasculature and cardiac function. While its impact on target organs has been extensively studied, little is known about its effects on highly vascularized organs, such as those from the male reproductive system. This study explores the impact of PAH on testis and epididymis, evaluating the potential role of combined exercise training as a non-pharmacological strategy to mitigate alterations in these organs. Methods: Male Wistar rats (n = 8/group) were assigned to one of three groups: sedentary control, sedentary PAH, and exercise PAH. PAH was induced by monocrotaline administration (60 mg Kg−1, i.p). The exercise PAH group underwent three weeks of combined physical training, including treadmill aerobic activity and resistance training on a ladder. Testis and epididymis were analyzed histologically, histomorphometrically, and biochemically for antioxidant activity, oxidative stress markers, and sperm parameters. Results: Sedentary PAH animals showed reductions in body and epididymis weight, normal seminiferous tubule percentage, and testicular morphometric parameters. These changes led to disorganized seminiferous tubules and compromised sperm production and sperm count in the testis and epididymis. Combined physical training improved testicular morphometric alterations and increased sperm count in hypertensive animals. Conclusions: PAH negatively affects testicular structure and function, leading to low sperm production. Combined physical training mitigated these effects by preserving testicular architecture and improving reproductive parameters, though it appeared less effective for the epididymis. These findings suggest physical training as a potential therapeutic strategy to protect reproductive health in PAH. Full article
(This article belongs to the Special Issue Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches)
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12 pages, 1736 KiB  
Article
The Importance of Dose Escalation in the Treatment of Pulmonary Arterial Hypertension with Treprostinil
by Piotr Kędzierski, Marta Banaszkiewicz, Michał Florczyk, Michał Piłka, Rafał Mańczak, Maria Wieteska-Miłek, Piotr Szwed, Krzysztof Kasperowicz, Katarzyna Wrona, Szymon Darocha, Adam Torbicki and Marcin Kurzyna
Biomedicines 2025, 13(1), 172; https://doi.org/10.3390/biomedicines13010172 - 13 Jan 2025
Viewed by 562
Abstract
Background: Treprostinil, which is administered via continuous subcutaneous or intravenous infusion, is a medication applied in the treatment of pulmonary arterial hypertension (PAH). The dose of treprostinil is adjusted on an individual basis for each patient. A number of factors determine how [...] Read more.
Background: Treprostinil, which is administered via continuous subcutaneous or intravenous infusion, is a medication applied in the treatment of pulmonary arterial hypertension (PAH). The dose of treprostinil is adjusted on an individual basis for each patient. A number of factors determine how well patients respond to treatment. Objectives: The aim of this study was to identify factors that may influence the clinical response to the dose of treprostinil at 3 months after the start of therapy. Methods: The factors influencing treatment response were analyzed in consecutive PAH patients who started receiving treprostinil treatment. The treatment efficacy was assessed as improvement in 6 min walk distance (6MWD) and WHO functional class (WHO FC), a reduction in N-terminal prohormone of brain natriuretic peptide (NTproBNP), and the percentage of patients achieving low-risk status after 12 months of treatment. Results: A total of 83 patients were included in this analysis. Classification of patients according to the tertiles of treprostinil dose achieved at 3 months after drug inclusion shows that after 12 months of follow-up, the median WHO FC in the highest dose group was lower than that in the intermediate dose group (WHO FC II vs. WHO FC III, p = 0.005), the median NTproBNP was lower (922 pg/mL, vs. 1686 pg/mL, p = 0.036) and 6MWD was longer (300 m vs. 510 m, p = 0.015). The French Noninvasive Criteria (NIFC) scale score was higher (2 vs. 0, p = 0.008), and the Reveal scale score was lower (5.0 vs. 8.5, p = 0.034). In the group of patients who exceeded a dose of 19.8 ng/kg/min within 3 months, an improvement in 6MWD was observed significantly more often after one year of therapy, and they were more likely to show an increase in NIFC scale scores after one year of therapy than the group of patients who received the lower dose (65% vs. 30%, p = 0.02). In the group of patients younger than 50 years of age, a statistically significant correlation was observed between the dose of treprostinil achieved after three months of treatment and the parameters assessed after 12 months of treatment, including WHO FC, 6MWD, and NIFC prognostic scale scores (all p < 0.05). Conclusions: The clinical effect of treatment is critically dependent on the rapid escalation of the treprostinil dose during the first three months of treatment. Full article
(This article belongs to the Special Issue Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches)
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Review

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18 pages, 1432 KiB  
Review
Emerging Mechanistic Insights and Therapeutic Strategies for Pulmonary Arterial Hypertension: A Focus on Right Ventricular Dysfunction and Novel Treatment Pathways
by Masab Mansoor and Andrew Ibrahim
Biomedicines 2025, 13(3), 600; https://doi.org/10.3390/biomedicines13030600 - 1 Mar 2025
Viewed by 197
Abstract
Background/Objectives: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder characterized by increased pulmonary vascular resistance, right ventricular dysfunction, and high mortality rates. Despite advancements in vasodilatory therapies, PAH remains a life-threatening condition with limited curative options. This review aimed to explore [...] Read more.
Background/Objectives: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder characterized by increased pulmonary vascular resistance, right ventricular dysfunction, and high mortality rates. Despite advancements in vasodilatory therapies, PAH remains a life-threatening condition with limited curative options. This review aimed to explore emerging molecular mechanisms, novel therapeutic targets, and future research directions in PAH treatment, focusing on strategies to improve long-term patient outcomes. Methods: This review synthesized recent advancements in PAH pathophysiology and therapeutic development. A structured literature search was conducted on PubMed and ClinicalTrials.gov using keywords such as “Pulmonary Arterial Hypertension”, “vascular remodeling”, “metabolic dysfunction”, and “emerging therapies”. Studies published between 2015 and 2025 were included, with a focus on preclinical models, clinical trials, and translational research. Key areas of investigation include vascular remodeling, metabolic dysregulation, inflammation, and right ventricular dysfunction. The review also evaluated the potential of novel pharmacological agents, gene-based therapies, and AI-driven diagnostics for PAH management. Results: Recent studies highlight dysregulated BMPR2 signaling, epigenetic modifications, and inflammatory cytokine pathways as critical contributors to PAH progression. Emerging therapies such as JAK-STAT inhibitors, metabolic reprogramming agents, and mesenchymal stromal cell-derived extracellular vesicles (EVs) show promise in preclinical and early clinical trials. Additionally, AI-enhanced imaging and non-invasive biomarkers are improving PAH diagnostics. Future research directions emphasize precision medicine approaches and the development of RV-targeted therapies. Conclusions: PAH remains a complex and fatal disease requiring multifaceted therapeutic strategies beyond traditional vasodilation. Advances in molecular-targeted treatments, AI-driven diagnostics, and personalized medicine offer new hope for disease-modifying interventions. Future research must bridge translational gaps to bring novel therapies from bench to bedside, improving survival and quality of life in PAH patients. Full article
(This article belongs to the Special Issue Pulmonary Arterial Hypertension: From Molecular Basis to Therapeutic Approaches)
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