Research progress on the pathogenesis of CTEPH

Heart Fail Rev. 2019 Nov;24(6):1031-1040. doi: 10.1007/s10741-019-09802-4.


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.

Keywords: Pathogenesis; Pulmonary hypertension; Thromboembolism.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Animals
  • Blood Coagulation Disorders / physiopathology
  • Chronic Disease
  • Endarterectomy / adverse effects*
  • Fibrinolysis / physiology
  • Genetic Predisposition to Disease / genetics
  • Humans
  • Hypertension, Pulmonary / complications*
  • Inflammation / physiopathology
  • Mice
  • Models, Animal
  • Neovascularization, Pathologic / physiopathology
  • Prognosis
  • Prospective Studies
  • Pulmonary Artery / physiopathology*
  • Pulmonary Embolism / epidemiology
  • Pulmonary Embolism / mortality
  • Pulmonary Embolism / physiopathology*
  • Pulmonary Embolism / surgery
  • Risk Factors
  • Vascular Remodeling / physiology