Learning and research

Pulmonary arterial hypertension (PAH) is a chronic, progressive, and ultimately life-threatening disease characterized by vascular remodeling and increasing pulmonary vascular resistance. Despite significant advances in diagnostic tools and therapeutic strategies, clinical intervention often begins only when patients become symptomatic—typically at New York Heart Association (NYHA) functional class II or higher.

In this PH Community Call, we discussed:

• Treatment Response and Survival in Methamphetamine‐Associated Pulmonary Arterial Hypertension by Cole Uhland and Franz P Rischard

Children with single ventricle heart disease (SVHD) demonstrate decreased arginine/NO metabolism following Stage 2 (Glenn) palliation, associated with poor postoperative outcomes. It is unknown if arginine dysregulation persists at Stage 3 (Fontan). 

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but severe complication of pulmonary embolism (PE), yet its underlying mechanisms remain poorly understood. Peptidyl-prolyl cis/trans isomerase (Pin1), a regulatory enzyme involved in thrombosis, inflammation, and vascular remodeling, may contribute to pulmonary vascular disease. 

Macitentan 10 mg and tadalafil 40 mg single-tablet combination therapy (M/T STCT) has been evaluated in the global A DUE study (NCT03904693). Here, we report the results of a subgroup analysis in participants from China.

Pulmonary arterial hypertension (PAH) is a fatal disease with limited available treatments and is characterized by pulmonary vascular remodeling. Substance P (SP) may be involved in vascular remodeling in patients with PAH. However, the underlying mechanism is currently unknown. 

Severe diffusion impairment in pulmonary arterial hypertension (PAH), particularly in idiopathic PAH (IPAH), has garnered considerable attention. However, comprehensive data on low diffusion capacity of the lungs for carbon monoxide (DLCO) with preserved lung function remain limited in broader pulmonary hypertension (PH) cohorts. 

During the COVID-19 pandemic, Brazil was one of the most affected countries. Patients presented higher risk of acute venous thromboembolism (VTE), in particular, pulmonary embolism (PE).

The American Lung Association and Pulmonary Hypertension Association convened a scientific roundtable of pulmonary hypertension experts to discuss the latest recommendations from the European Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (PH) and from the 7th World Symposium on Pulmonary Hypertension (WSPH).