Heart Failure Reviews

, Volume 24, Issue 6, pp 1031–1040 | Cite as

Research progress on the pathogenesis of CTEPH

  • Lu Yan
  • Xin Li
  • Zhihong LiuEmail author
  • Zhihui Zhao
  • Qin Luo
  • Qin Zhao
  • Qi Jin
  • Xue Yu
  • Yi Zhang


Chronic thromboembolic pulmonary hypertension (CTEPH) is an established long-term complication of pulmonary thromboembolism (PTE). However, studies have shown that many patients with a definitive CTEPH diagnosis have no history of symptomatic PTE, suggesting that PTE is not the only cause of CTEPH. Despite extensive progress in research on pulmonary hypertension in recent years, due to a lack of relevant studies on the pathophysiology of CTEPH, implementing pulmonary endarterectomy (PEA) in patients has many challenges, and the prognosis of patients with CTEPH is still not optimistic. Therefore, revealing the pathogenesis of CTEPH would be of great significance for understanding the occurrence and development of CTEPH, developing relevant drug treatment studies and formulating intervention strategies, and may provide new preventive measures. This article summarizes the current research progress in CTEPH pathogenesis from the perspective of risk factors related to medical history, abnormal coagulation and fibrinolytic mechanisms, inflammatory mechanisms, genetic susceptibility factors, angiogenesis, in situ thrombosis, vascular remodeling, and other aspects.


Thromboembolism Pulmonary hypertension Pathogenesis 


Compliance with ethical standards

Conflict of interest

The authors have no conflicts of interest to declare.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Lu Yan
    • 1
  • Xin Li
    • 1
  • Zhihong Liu
    • 1
    Email author
  • Zhihui Zhao
    • 1
  • Qin Luo
    • 1
  • Qin Zhao
    • 1
  • Qi Jin
    • 1
  • Xue Yu
    • 1
  • Yi Zhang
    • 1
  1. 1.Center for Pulmonary Vascular Diseases, Fuwai Hospital, National Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

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