In 1979, during my cardiology fellowship, a 16-year-old with primary pulmonary hypertension (PPH), a rare and severe condition at that time, spurred my research. There was limited literature then. My pediatric cardiology colleagues, who were experienced in dealing with pulmonary hypertension linked to congenital heart diseases, introduced me to vasoreactivity testing, a method involving the administration of oxygen and tolazoline to assess operability. Tolazoline administration resulted in a significant decrease in pulmonary artery (PA) pressure, suggesting potential therapeutic responsiveness, though diuretics were the only treatment for pulmonary hypertension and right heart failure at that time. This prompted my search for a viable solution.

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. 

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent obstruction and vascular remodeling of the pulmonary arteries following pulmonary thromboembolism (PTE), diagnosed after a minimum of 3 months of therapeutic anticoagulation. 

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.

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 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.

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. 

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.

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 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).