Pulmonary hypertension is a rare and complex disease that arises from a wide range of underlying conditions. Therapeutic options have expanded enormously over the last two decades resulting in major improvements in prognosis for some patients. As a consequence, it is vital that the disease is recognised early and referred on to specialist centres for further investigation and definitive diagnosis to improve prognosis in this life-altering condition. Unfortunately, it remains the case in the UK that there is a delay in the diagnosis of pulmonary hypertension, often months or years after the onset of symptoms. This review aims to highlight key points in initial management and referral of patients with suspected pulmonary hypertension and presents three cases to underline these areas.
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http://dx.doi.org/10.4997/JRCPE.2021.419 | DOI Listing |
NPJ Syst Biol Appl
January 2025
United Therapeutics Corporation, Silver Spring, MD, USA.
Challenges in drug development for rare diseases such as pulmonary arterial hypertension can be addressed through the use of mathematical modeling. In this study, a quantitative systems pharmacology model of pulmonary arterial hypertension pathophysiology and pharmacology was used to predict changes in pulmonary vascular resistance and six-minute walk distance in the context of oral treprostinil clinical studies. We generated a virtual population that spanned the range of clinical observations and then calibrated virtual patient-specific weights to match clinical trials.
View Article and Find Full Text PDFJ Rheumatol
January 2025
Florenzo Iannone, Rheumatology Unit - Department of Precision and Regenerative Medicine of Jonian Area University of Bari, Bari, Italy.
Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.
Circ J
January 2025
Department of Echo-imaging Center, Aizawa Hospital.
Background: Selexipag, an oral prostacyclin (PGI) receptor agonist, is approved for adult patients with pulmonary arterial hypertension (PAH). This study evaluated the efficacy and safety of selexipag for Japanese pediatric patients with PAH.
Methods And Results: The study enrolled 6 patients who received selexipag twice daily at an individualized dose based on body weight; maintenance doses were determined for each patient by 12 weeks after starting administration.
Phytomedicine
December 2024
Univ Coimbra, Coimbra Institute for Clinical and Biomedical Research (iCBR), Faculty of Medicine, Azinhaga de S. Comba, Coimbra 3000-548, Portugal; Univ Coimbra, Center for Innovative Biomedicine and Biotechnology (CIBB), Coimbra, Portugal; Clinical Academic Centre of Coimbra (CACC), Coimbra, Portugal.
Background: Pulmonary Arterial Hypertension (PAH) is characterized by pulmonary vascular remodelling, often associated with disruption of BMPR2/Smad1/5 and BMPR2/PPAR-γ signalling pathways that ultimately lead to right ventricle failure. Disruption of intercellular junctions and communication and a pro-angiogenic environment are also characteristic features of PAH. Although, current therapies improve pulmonary vascular tone, they fail to tackle other key pathological features that could prevent disease progression.
View Article and Find Full Text PDFJ Bras Pneumol
January 2025
. Programa de Pós-Graduação em Pneumologia, Universidade Federal do Rio Grande do Sul, Porto Alegre (RS) Brasil.
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