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29 July 2025

Pulmonary hypertension (PH) carries a poor prognosis and a high mortality. Loss of pulmonary arterial compliance (PAC) plays a significant role in the development of PH and is an early predictor of mortality. Currently, there are no therapeutic options to overcome the loss of PAC. Aria CV (Saint Paul, MN) has developed a device to augment PAC. The device consists of a 20-cc balloon and anchor that can be implanted in the pulmonary artery using a minimally invasive procedure, a catheter, and a gas reservoir.

Pulmonary Circulation
29 July 2025

Iron deficiency (ID) is prevalent in pulmonary hypertension(PH), but there is no consensus on ID definition and its possible correlation to prognostic markers. Hence, in this study, PH-patients were recruited at the University Hospital Zurich from May 2019 to April 2021. Clinical and hemodynamic characteristics were recorded at inclusion and venous blood samples were taken. 

Pulmonary Circulation
29 July 2025

This dynamic session featured focused presentations on mechanical support strategies for paediatric bridge to lung transplant across the lifespan—from infancy to adolescence—highlighting how each expert’s practice has evolved, and concluding with interactive, case-based discussions.

IDDI Workstream or Task Force Learning
28 July 2025

Awareness of health disparities that exist across different self-identified racial and ethnic groups are essential to developing interventions that improve the quality of care of patients with rare diseases such as pulmonary arterial hypertension (PAH). We sought to determine whether there are important differences in clinical characteristics and illness severity at the time of PAH diagnosis among different racial/ethnic groups.

Pulmonary Circulation
27 July 2025

Pulmonary arterial hypertension (PAH) is a disorder characterized by progressive remodeling of small pulmonary arteries, leading to increased pulmonary vascular resistance, right ventricular failure and premature death (1-2). Over the past 30 years, significant advancements have been made in the treatment of PAH, including the recent approval of sotatercept, a first-in-class fusion protein that acts as a ligand trap for activins and growth differentiation factors, which are key players in the transforming growth factor β (TGF-β) superfamily (3-4).

Pulmonary Circulation
27 July 2025

To evaluate current digital inclusion in the Scottish pulmonary hypertension population, a paper questionnaire was offered to the entirety of patients with pulmonary arterial hypertension in Scotland. The Scottish Index of Multiple Deprivation was used to stratify patients into deprivation deciles. 464 patients returned questionnaires (86%). 91% had reliable internet access. 89% had access to an internet-enabled device. 

Pulmonary Circulation
23 July 2025

Letter to the editor: We read with great interest the recent publication by de la Fuente et al. describing the effects of oral melatonin therapy on quality of life, oxidative stress, and cardiovascular function in patients with World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH) [1]. This pilot study represents an important step forward in exploring novel therapeutic approaches for PAH, particularly those addressing oxidative stress as a key pathophysiological mechanism. We would like to offer additional perspectives that may enhance the clinical significance of these findings [2].

Pulmonary Circulation
18 July 2025

Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6 M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008–2021 were used. 

Pulmonary Circulation
16 July 2025

Pulmonary arterial hypertension (PAH) is a progressive vascular disease characterized by small artery occlusion, increased pulmonary vascular resistance, and right heart failure. HSPH1, a member of the heat shock protein family, has been shown to inhibit protein aggregation but its role in PAH remains unclear. The purpose of this study was to explore the expression pattern and potential mechanism of HSPH1 in PAH, and to provide new diagnostic markers for PAH.

Pulmonary Circulation
16 July 2025

The objective of this study is to assess the effect of subcutaneous treprostinil (TRE) administered peri-operatively after Fontan operation on chest tube duration (CTD), hospital length of stay (LOS), and post-operative hemodynamics. This is a single center randomized, blinded, placebo-controlled study of pediatric patients with single ventricle congenital heart disease undergoing Fontan operation between September 2015 and September 2019. 

Pulmonary Circulation