Sex Matters: Translational Advances in Respiratory and PAH Research

Eisenmenger syndrome (ES) represents an advanced stage of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is characterized by elevated pulmonary vascular resistance, with a right-to-left shunt and resting hypoxemia. ES is associated with high morbidity and mortality. 

Portopulmonary hypertension (PoPH) is a subtype of pulmonary arterial hypertension (PAH) associated with portal hypertension, occurring in 2%–6% of cirrhotic patients. Although guidelines recommend routine echocardiography before transplantation, the prevalence, and outcomes of PAH-specific therapy in cirrhotic patients remain unclear.

Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is one of the most frequent etiologies of pulmonary arterial hypertension in Brazil and globally. The disease is a complication of hepatosplenic schistosomiasis characterized by portal hypertension, egg deposition, and granuloma formation in the pulmonary arteries leading to inflammation, vascular remodeling, increased pulmonary vascular resistance, and progressive right heart failure. 

Right ventricle (RV) dysfunction is central to pulmonary arterial hypertension (PAH) yet its molecular mechanisms remain unclear. This study aimed to identify biomarkers and therapeutic targets for RV remodeling in PAH.