Purpose of Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of acute pulmonary embolism (PE), in which the red, platelet-rich thrombus does not resolve but forms into an organized yellow, fibrotic scar-like obstruction in the pulmonary vasculature. Here we review the pathobiology of CTEPH.
Our current knowledge has predominantly been informed by studies of human samples and animal models that are inherently limited in their ability to recapitulate all aspects of the disease. These studies have identified alterations in platelet biology and inflammation in the formation of a scar-like thrombus that comprised endothelial cells, myofibroblasts, and immune cells, along with a small vessel pulmonary arterial hypertension-like vasculopathy.
The development of CTEPH-specific therapies is currently hindered by a limited knowledge of its pathobiology. The development of new CTEPH medical therapies will require new insights into its pathobiology that bridge the gap from bench to bedside.
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Circulating endothelial cells
Chronic thromboembolic pulmonary hypertension
Deep venous thrombosis
Endothelial progenitor cells
Inferior Vena Cava
Mean pulmonary arterial pressure
Pulmonary arterial hypertension
Platelet endothelial cell adhesion molecule-1
Pulmonary vascular resistance
Transforming growth factor β
Tumor necrosis factor α
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Conflict of Interest
Dr. Tapson reports grants from Bayer, and grants and personal fees from Janssen and Actelion, outside the submitted work.
Dr. Maron reports personal fees from Actelion, outside the submitted work. In addition, Dr. Maron has a patent US patent 9,605,047 issued, a patent US pending patent PCT/US2019/059890 pending, and patent applications 62475955 and 029672 pending.
Dr. Rajagopal reports grants and personal fees from Janssen and United Therapeutics, and personal fees from Altavant, Apie Therapeutics, Bayer, Insmed, and Liquidia Technologies, outside the submitted work. In addition, Dr. Rajagopal has a patent US Patent 62/673,175. “Dynamic 129Xe Gas Exchange Spectroscopy” licensed to Polarean Corporation.
The other authors declare that they have no conflict of interest.
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Alba, G.A., Atri, D., Darbha, S. et al. Chronic Thromboembolic Pulmonary Hypertension: the Bench. Curr Cardiol Rep 23, 141 (2021). https://doi.org/10.1007/s11886-021-01572-6