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Pulmonary Hypertension: Molecular Diagnosis, Pathogenesis, Biomarkers and Therapies

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (20 October 2025) | Viewed by 2588

Special Issue Editor


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Guest Editor
Cardiology Unit, University Policlinic Hospital Riuniti, Viale Luigi Pinto 1, 71122 Foggia, Italy
Interests: heart failure; pulmonary hypertension; pulmonary arterial hypertension; cardiomyopathies; cardiovascular medicine

Special Issue Information

Dear Colleagues,

Pulmonary arterial hypertension (PAH) is a rare condition, characterized by high pulmonary artery pressure, leading to right ventricular (RV) dysfunction and potential life-threatening consequences. PAH involves complex mechanisms: vasoconstriction, vascular remodeling, endothelial dysfunction, inflammation, oxidative stress, fibrosis, RV remodeling, metabolic imbalance, and thrombosis. These mechanisms are mediated by several pathways, like nitric oxide and prostacyclin.

PAH falls into Group 1 of pulmonary hyperthension (PH), with specific therapies. Early and accurate diagnosis is crucial and requires clinical evaluation and right heart catheterization. Mortality rates remain high, despite the development of new classes of drugs and the recent discovery of new pathways.

This Special Issue aims to delve deeper into molecular diagnosis, pathogenesis, biomarkers and therapies of PH, presenting the main innovations on biomolecular experiments, molecular mechanisms and pathophysiology underlying the development of PH, as well as new pharmacological discoveries to counteract this condition.

Dr. Michele Correale
Guest Editor

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Keywords

  • pulmonary hypertension
  • pulmonary arterial hypertension
  • pulmonary vascular disease
  • pulmonary circulation
  • cor pulmonale
  • chronic lung disease

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Published Papers (2 papers)

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Research

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15 pages, 3300 KB  
Article
Effects of Dietary Terpinen-4-ol on Oxidative Stress and Mitochondrial Biogenesis in the Liver of Broilers with Pulmonary Hypertension Syndrome
by Xinyue Jiang, Liang Fei, Yayun Yang, Jiao Han, Zhaoxin Tang, Jianzhao Liao, Lianmei Hu, Ying Li and Jiaqiang Pan
Int. J. Mol. Sci. 2025, 26(16), 7702; https://doi.org/10.3390/ijms26167702 - 9 Aug 2025
Viewed by 583
Abstract
Pulmonary hypertension syndrome (PHS), a metabolic disorder causing economic losses in broilers, arises from hypoxia-induced portal hypertension and liver cirrhosis, triggering mitochondrial oxidative damage, excessive ROS production, and altered mitochondrial biogenesis. This study explored terpinen-4-ol (T4O), known for antimicrobial and anti-inflammatory properties, in [...] Read more.
Pulmonary hypertension syndrome (PHS), a metabolic disorder causing economic losses in broilers, arises from hypoxia-induced portal hypertension and liver cirrhosis, triggering mitochondrial oxidative damage, excessive ROS production, and altered mitochondrial biogenesis. This study explored terpinen-4-ol (T4O), known for antimicrobial and anti-inflammatory properties, in mitigating PHS. Broilers were divided into four groups, including PHS-affected birds with/without T4O supplementation. Analyses revealed that PHS birds exhibited reduced antioxidant capacity, elevated MDA and ROS levels, increased mitochondrial numbers, and upregulated expression of oxidative stress markers (Keap1, HO-1, Nrf-2) and mitochondrial biogenesis regulators (PGC-1α, Nrf-1, Tfam). T4O administration enhanced antioxidant activity, reduced ROS and MDA, suppressed compensatory mitochondrial proliferation, and downregulated Keap1/Nrf-2 and mitochondrial biogenesis pathways. These effects suggest that T4O alleviates hypoxia-driven oxidative stress and mitochondrial dysfunction in broilers. Findings highlight T4O’s potential as a therapeutic agent to mitigate PHS-related losses in poultry production. Full article
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Review

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26 pages, 1050 KB  
Review
Pathophysiology of Pulmonary Arterial Hypertension: Focus on Vascular Endothelium as a Potential Therapeutic Target
by Michele Correale, Valentina Mercurio, Ester Maria Lucia Bevere, Beatrice Pezzuto, Lucia Tricarico, Umberto Attanasio, Angela Raucci, Anne Lise Ferrara, Stefania Loffredo, Claudio Puteo, Massimo Iacoviello, Maurizio Margaglione, Natale Daniele Brunetti, Carlo Gabriele Tocchetti, Piergiuseppe Agostoni, Claudio Mussolino and Maria Cristina Vinci
Int. J. Mol. Sci. 2025, 26(19), 9631; https://doi.org/10.3390/ijms26199631 - 2 Oct 2025
Viewed by 1626
Abstract
Pulmonary arterial hypertension (PAH) is a rare condition characterized by high pulmonary artery pressure leading to right ventricular dysfunction and potential life-threatening consequences. It primarily affects the pre-capillary pulmonary vascular system. The exact pathophysiological mechanisms underlying PAH are not entirely known. Environmental factors; [...] Read more.
Pulmonary arterial hypertension (PAH) is a rare condition characterized by high pulmonary artery pressure leading to right ventricular dysfunction and potential life-threatening consequences. It primarily affects the pre-capillary pulmonary vascular system. The exact pathophysiological mechanisms underlying PAH are not entirely known. Environmental factors; genetic predisposition; mitochondrial and microRNA dysfunction; and inflammatory, metabolic, and hormonal mechanisms may be involved. A central role is played by the dysfunction of the pulmonary vascular endothelium. This alteration is characterized by a reduction in vasodilatory and antiproliferative factors such as prostacyclin and nitric oxide and an increase in vasoconstrictive and mitogenic substances such as endothelin and thromboxane A2. Such imbalance leads to a progressive increase in pulmonary vascular resistance. The aim of the present review is to focus on the vascular endothelium and its role as a potential therapeutic target in PAH. Full article
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