Should we be treat CTEPD with no PH in the multimodal treatment era?

10 February 2026

Presented at PVRI 2026 Congress by Irene Lang, Medical University Vienna

Summary

Chronic thromboembolic pulmonary disease (CTEPH) is a significant condition that requires careful management. It is crucial to understand its prevalence and treatment options, including pulmonary endarterectomy and balloon pulmonary angioplasty. Accurate definitions and classifications are essential for effective patient management. Individualised care can lead to better outcomes, and the discussion includes emerging concepts and the clinical relevance of different phenotypes of chronic thromboembolism.

 

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Our speaker

Irene Lang, Medical University Vienna
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Transmembrane Protein 100 Expression on Endothelial Cells Vascularizing Thrombi in Chronic Thromboembolic Pulmonary Hypertension Modulates TGFβ1−ALK1 Signaling During Angiogenesis

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.

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