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.
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.
This webinar explores Group 3 pulmonary hypertension (PH) associated with COPD and ILD, including updated definitions, diagnosis, and treatment approaches.
Ten patients with post-COVID ILD-PH are described. This complication occurred only in patients after prolonged intensive care (ICU) admission for invasive mechanical ventilation.
Pregnancy in pulmonary arterial hypertension (PAH) is associated with substantial risks. Endothelin receptor antagonists (ERAs), including macitentan and bosentan, are contraindicated in pregnancy due to teratogenic effects observed in animal studies.
