Long-term intravenous (IV) epoprostenol is a key therapy for pulmonary arterial hypertension (PAH); however, rare immune-mediated conditions occurring during therapy remain incompletely characterised.
Reperfusion pulmonary edema (RPE) is a severe complication after pulmonary endarterectomy (PEA) and is associated with prolonged mechanical ventilation, organ dysfunction, and worse outcome.
Patients diagnosed with intermediate high-risk pulmonary embolism (IHRPE) are at significant risk for clinical deterioration during hospitalization; however, clinical tools to identify which patients will worsen are imprecise.
Pulmonary Langerhans cell histiocytosis (PLCH) frequently complicated by pulmonary hypertension (PH), which markedly worsens prognosis. We retrospectively reviewed three institutional PLCH-PH cases treated with off-label Sotatercept added to background triple therapy and performed a systematic review of published PLCH-PH reports (PubMed/Embase through May 2025).