Learning and research

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 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.

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. 

Interstitial lung disease (ILD) complicated by pulmonary hypertension (PH) is associated with poor outcomes. However, real-world data characterizing the hemodynamic profiles of ILD patients undergoing right heart catheterization (RHC) remain limited. We retrospectively analyzed ILD patients who underwent RHC between 2006 and 2024.

As with adult pulmonary hypertension (PH), high morbidity and mortality persist with diverse types of paediatric PH. Despite major advances in pharmacologic therapies based on extensive studies in adult PH, few drugs have been comprehensively studied in neonates, infants, and children, leaving current paediatric PH care largely dependent on small observational studies and extrapolation of evidence from adult clinical trials.

Studies have shown sex differences in the outcomes of chronic thromboembolic pulmonary hypertension (CTEPH) and in patients who have undergone pulmonary endarterectomy. Limited data exist regarding outcomes of balloon pulmonary angioplasty (BPA) for female versus male subjects. In this analysis cohort, a total of 767 sessions of BPA were performed on 144 patients of which 83 (58%) were female. There were no differences in age, body mass index and comorbidities between males and females.

In a recent study conducted by Laura Stourm et al., the authors identified that patients with pulmonary hypertension (PH) harboring loss-of-function variants in the Filamin A (FLNA) gene exhibit a unique spectrum of phenotypes. These include pulmonary involvement with lung parenchymal abnormalities and emphysema, as well as a range of extrapulmonary manifestations such as dysmorphic facial features, epilepsy, congenital heart defects, valvular and aortic diseases, thrombocytopenia, and periventricular nodular heterotopia (PVNH).

2025 Select Abstracts from the 18th Annual International Conference on Neonatal and Childhood Pulmonary Vascular Disease

Portopulmonary hypertension (PoPH), a type of pulmonary arterial hypertension (PAH) in patients with liver disease, is associated with high morbidity and mortality. The relationship between cardiopulmonary hemodynamics, PAH therapy, and survival in PoPH remains unclear.

Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival.