Sotatercept to Wean Off Prostacyclin Infusion Therapy for Pulmonary Arterial Hypertension: A Case Report

Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, proliferation, fibrosis, and microthrombosis of the pulmonary vasculature, which causes elevated pulmonary arterial pressure and vascular resistance leading to right ventricular failure and death.

This exploratory analysis assessed whether plasma biomarkers predict the response to switching from phosphodiesterase type 5 inhibitors (PDE5is) to the soluble guanylate cyclase stimulator riociguat in patients with pulmonary arterial hypertension. Selected biomarkers at baseline and their changes to Week 24 were evaluated in patients with and without a favorable response to riociguat in two trials: RESPITE, in which patients with an inadequate response to PDE5i were switched to riociguat; and REPLACE, in which patients at intermediate risk of 1-year mortality despite a PDE5i were randomized to remain on PDE5i or were switched to riociguat.

Portopulmonary hypertension (PoPH), a type of pulmonary arterial hypertension (PAH) in patients with liver disease, is associated with high morbidity and mortality. The relationship between cardiopulmonary hemodynamics, PAH therapy, and survival in PoPH remains unclear.

Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival.