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).
Endothelial cells within chronic pulmonary artery thrombi in CTEPH overexpress transmembrane protein 100 (TMEM100), an activin A receptor-like kinase 1 (ACVRL1 or ALK1) signaling-dependent gene, and TGFβ1 upregulated TMEM100 transcription in healthy lung ECs. TMEM100 permitted the TGFβ1-induced increase of ALK1, while repressing ALK5, and preventing ALK1–TMEM100 signaling impaired angiogenesis ex vivo. Our data indicate that TGFβ1–ALK1–TMEM100 signaling is active during CTEPH thrombus revascularization.
The optimal treatment strategy for patients with acute intermediate–high-risk pulmonary embolism (PE) remains uncertain. This randomized clinical trial (PRETHA) aimed to evaluate the efficacy and safety of percutaneous reperfusion therapies—trans-catheter thrombectomy and trans-catheter thrombolysis—compared with standard anticoagulation therapy.
A major consequence of acute exacerbation of chronic obstructive pulmonary disease (AECOPD) is pulmonary hypertension (PH), which raises morbidity and mortality rates. This study assessed biomarker profiles, clinical characteristics and diagnostic efficacy of important coagulation and inflammatory markers in AECOPD patients who have varying degrees of PH severity.
Pulmonary hypertension (PH) causes progressive pulmonary vascular resistance and right heart failure. We investigated whether beta-alanine (β-Ala) improves right ventricular (RV) remodeling and dysfunction in a monocrotaline (MCT)-induced PH rat model.
Exercise testing has long been essential for evaluating diagnosis, prognosis, and functional status in pulmonary hypertension (PH). Recent advances have clarified its role in defining reference values and prognostic markers. Nonetheless, substantial knowledge gaps persist regarding the implementation of invasive cardiopulmonary exercise testing (iCPET) and its potential to inform pathophysiology and therapeutic decision-making.
