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3 August 2025

Pulmonary arterial hypertension (PAH) in intermediate-risk patients poses a challenge for clinicians, particularly in determining the optimal timing for escalating pharmacological treatment. We conducted a multicenter cross-sectional study that analyzed data from the Colombian Pulmonary Hypertension Network (HAPredCO) on patients diagnosed with PAH. 

Pulmonary Circulation
1 August 2025

Pulmonary tumor thrombotic microangiopathy (PTTM) is a severe and fatal disease that rapidly causes pulmonary hypertension (PH). While imatinib shows potential for treating PH related to PTTM, it remains unclear if imatinib should continue or be discontinued after improvement in PH. Here, we present a case with PTTM in which PH was controlled for 9 months on imatinib, but recurred immediately after discontinuing of imatinib.

Pulmonary Circulation
30 July 2025

Segmental pulmonary hypertension (PH) in congenital heart disease remains poorly understood with data limited to case studies. We performed a retrospective, single center study in children treated with PH medications after unifocalization/pulmonary artery reconstruction for major aortopulmonary collaterals (MAPCA). Drug response was determined by hemodynamic changes across at least two cardiac catheterizations.

Pulmonary Circulation
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