Sotatercept: A New Era in Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by proliferative remodeling and obliterative narrowing of the pulmonary vasculature. While outcomes have improved with existing treatments targeting 3 main pathways, there remains a critical need for novel therapies that address different and novel mechanisms of PAH. Sotatercept, recently Food and Drug Administration (FDA) approved, is a groundbreaking fusion protein that binds to activin and growth differentiation factors, rebalancing antiproliferative and pro-proliferative signals to reverse remodeling in both the pulmonary vasculature and the right ventricle.

Acute Kidney Injury After Pulmonary Thromboendarterectomy: Associated Factors and Impact.

Background: Acute kidney injury (AKI) is a common complication after pulmonary thromboendarterectomy (PTE) that has been shown to be associated with worse outcomes. Our study assesses factors associated with the development of postoperative AKI after PTE and its impact on clinical outcomes.

Methods: We retrospectively analyzed 247 patients who underwent PTE at our institution between June 2009 and December 2020.

Clinical safety, pharmacokinetics, and efficacy of ambrisentan therapy in children with pulmonary arterial hypertension.

Recent trials in adult PAH revealed the efficacy of ambrisentan. However, in children with PAH, the clinical safety and pharmacokinetics of ambrisentan has not been well studied. Our aim was to investigate the clinical safety, pharmacokinetics, tolerability, and efficacy of endothelin receptor antagonist therapy with ambrisentan in children with pulmonary arterial hypertension (PAH).

Common atrium and pulmonary vascular disease.

This report describes a case series of six patients with congenital common atrium who developed pulmonary vascular disease. Three developed early pulmonary vascular disease within their first year of life, while the others became symptomatic at ages 2, 5, and 17. Four of the six underwent surgical repair, and five of the six patients are being treated on targeted pulmonary hypertension therapy.

Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension.

Objectives: This study investigated the short- and long-term outcome of children with pulmonary arterial hypertension (PAH) treated with inhaled iloprost.

Background: Inhaled iloprost has been approved for the treatment of adults with PAH, but little is known about the effects in children with PAH.

Methods: We evaluated the acute effects of inhaled iloprost on hemodynamic status and lung function and the response to long-term therapy in 22 children (range 4.