Learning and research

Substantial evidence from animal models supports the concept of inhibiting peripheral serotonin synthesis for the treatment of pulmonary arterial hypertension (PAH), as demonstrated by pharmacological blockade or genetic deletion of tryptophan hydroxylase 1 (TPH1) [1-5]. Most recently, we have shown that daily inhalation of TPT-004, a novel class TPH1 inhibitor, can alleviate PAH in the Sugen-hypoxia (SuHx) rat model [1]. 

Phenotypic transition of pulmonary artery smooth muscle cells (PASMCs) under hypoxic conditions, which in turn causes increased proliferation and migration capacity, is an important pathological process in Hypoxic pulmonary hypertension (HPH). Although research on the phenotypic transition of PASMCs has been ongoing, little is known about the specific molecular mechanisms underlying this process.

Activin-A is elevated in pulmonary arterial hypertension (PAH) patients, and reportedly suppresses BMPR-II. This suggests one mechanism of action for PAH drug, sotatercept, an activin-ligand trap. However, we were unable to confirm that activin-A reduces BMPR-II in pulmonary endothelial cells. Thus, it seems unlikely that sotatercept influences BMPR-II or PAH via this mechanism.

The 2025 PVRI International Conference was held in Rio de Janeiro, Brazil under the theme “Embracing Heterogeneity” over 4 days of insightful discussions on pulmonary hypertension (PH). The conference covered a diverse array of topics spanning basic science, translational research, and clinical observations from around the world. Attendees included experts from backgrounds ranging from early-career investigators to senior scientists and clinicians and industry partners, representing 25 countries across 5 continents.

Fibrosing mediastinitis (FM) can block pulmonary vessels and airways, hindering treatment efficacy. Pulmonary artery (PA) stenting might provide a solution in such cases. This study involved 30 patients who had 49 PA stenting procedures for FM. Data on baseline characteristics, CT pulmonary angiography images, stent patency, and hemodynamics were collected.

This systematic literature review of three Western European Countries identified N = 48 different terms to describe pulmonary arterial pressure and N = 35 thresholds used to define pulmonary hypertension in published empiric research studies. There is an urgent need to standardize pulmonary artery pressure nomenclature and pulmonary hypertension definitions in clinical research reports.

The aim of the open label extension (OLE) of CTREPH study was to characterize multimodal treatment in patients with severe inoperable CTEPH, to describe long-term subcutaneous (SC) treprostinil safety and tolerability, and to evaluate change in functional class and exercise capacity over 24 months since completion of the blinded phase of CTREPH.

Bufei huoxue capsules (BFHX), manufactured products of traditional Chinese medicine, have demonstrated anti-inflammatory properties and efficacy against chronic pulmonary diseases and COVID-19. This study was designed to further determine the clinical efficacy of BFHX in diverse patient subgroups during the convalescent phase of COVID-19, extending upon previously reported findings from a multicenter randomized controlled trial.

Data about pulmonary arterial hypertension (PAH) patients living in low- and middle-income countries remain scarce. This study assessed prognostic factors associated with time to clinical worsening (CW) or death of a cohort of PAH patients in Minas Gerais, Brazil.