Learning and research

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. 

PH-ILD carries a poor prognosis, particularly with PVR > 5 WU. The INCREASE trial demonstrated the successful use of inhaled treprostinil, but slow uptitration delays the effects. Our study suggests outpatient IV dobutamine as interim support, improving contractility and reducing PVR during uptitration.

Hypopituitarism has been reported in patients receiving continuous infusions of prostaglandin I2 (PGI2) analogues for pulmonary hypertension (PH). However, these patients' clinical characteristics, treatment, and prognoses remain unclear. This retrospective multicentre study included 22 patients who developed hypopituitarism while on continuous PGI2 analogue infusion between 1999 and 2021.

Pulmonary hypertension (PH) is a life-threatening condition frequently associated with exertional dyspnea. It remains diagnostically challenging due to limitations in current screening modalities. While the 6-min walk test (6MWT) has been applied for risk stratification in confirmed PH, its potential role in screening remains unexplored.

Children with chronic diseases, including pulmonary hypertension (PH), have an increased risk of anxiety and depression (AD), impacting mental health (MH), and quality of life (QoL). We sought to characterize the prevalence of AD in pediatric PH and identify associated factors. We developed a prospective cross-sectional study with 10 Pediatric Pulmonary Hypertension Network (PPHNet) centers.

This study aims to understand healthcare providers' (HCPs) decision to adopt double combination therapy with ERA + PDE5i for pulmonary arterial hypertension (PAH), and to explore whether a single tablet combination therapy (STCT) might increase adoption practices. 195 US HCPs completed a survey evaluating their PAH treatment preferences.