Learning and research

Twelve patients with severe pulmonary hypertension (PH) who had a pulmonary artery-to-descending aorta (Potts) shunt were treated with the novel PH drug sotatercept for an average of 12 months.

Pulmonary arterial hypertension (PAH) patients frequently present with cardiometabolic comorbidities and experience substantial hematologic and cardiovascular events.

Pulmonary arterial hypertension (PAH) is characterized by excessive pulmonary vasoconstriction and vascular remodelling, with mutations in bone morphogenetic protein receptor type 2 (BMPR2) being the most common genetic alteration associated with the disease. 

Accurate measurement of cardiopulmonary hemodynamics and exercise capacity is vital for PAH diagnosis and management. Terminal digit bias in clinical measures introduces non-differential error.

Pediatric idiopathic pulmonary arterial hypertension (IPAH) refractory to maximal medical therapy is associated with high morbidity and mortality, and therapeutic options remain limited.

Sex differences in COPD patients with concomitant pulmonary hypertension remain unexplored.

Pulmonary hypertension (PH) is widely recognized as a disease driven by both vasoconstriction and vascular remodeling. Vasoconstriction is thought to play a predominant role in the early stages of PH, whereas the contribution of vascular remodeling increases as the disease progresses. However, clinical or histological evidence of this pathological progression has not been reported. 

Endothelial heterogeneity and plasticity play an important role in lung development, homeostasis, and pathology. In recent years, increasing evidence has demonstrated that endothelial dysfunction contributes to the progression of various lung diseases, such as ADRS, PF, PH, and lung developmental disorders.

Early identification of pulmonary vascular disease remains a major unmet need in systemic sclerosis. In this context, the study by Kagami and colleagues provides important insights into the role of exercise stress echocardiography in detecting early hemodynamic abnormalities.