Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: A current understanding

Irene M. Lang, Raffaele Pesavento, Diana Bonderman, Jason Yuan

Research output: Contribution to journalArticle

194 Citations (Scopus)

Abstract

All available evidence today indicates that chronic thromboembolic pulmonary hypertension (CTEPH) is primarily caused by venous thromboembolism, as opposed to primary pulmonary vascular in situ thrombosis. Both the initial magnitude of clot and pulmonary embolism (PE) recurrence may contribute to the development of CTEPH. Only few specific thrombophilic factors, such as phospholipid antibodies, lupus anticoagulant and elevated factor VIII, are statistically associated with CTEPH. A mechanistic view of CTEPH as a disease caused by obliteration of central pulmonary arteries by pulmonary emboli is too simplistic. Based on available data one may speculate that PE may be followed by a pulmonary vascular remodelling process modified by infection, immune phenomena, inflammation, circulating and vascular-resident progenitor cells, thyroid hormone replacement or malignancy. Both plasmatic factors (hypercoagulation, 'sticky' red blood cells, high platelet counts and uncleavable fibrinogens) and a misguided vascular remodelling process contribute to major vessel and small vessel obliteration. Endothelial dysfunction and endothelial- mesenchymal transition may be important, but their precise roles remain obscure. There exists no animal model for CTEPH; therefore, experimentation in the future must include human tissues and clinical data in parallel.

Original languageEnglish (US)
Pages (from-to)462-468
Number of pages7
JournalEuropean Respiratory Journal
Volume41
Issue number2
DOIs
StatePublished - Feb 1 2013
Externally publishedYes

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Pulmonary Hypertension
Pulmonary Embolism
Lung
Blood Vessels
Lupus Coagulation Inhibitor
Venous Thromboembolism
Factor VIII
Embolism
Platelet Count
Thyroid Hormones
Fibrinogen
Pulmonary Artery
Phospholipids
Thrombosis
Stem Cells
Animal Models
Erythrocytes
Inflammation
Recurrence
Antibodies

Keywords

  • Chronic thromboembolic pulmonary hypertension
  • Coagulation
  • Review
  • Vascular biology

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension : A current understanding. / Lang, Irene M.; Pesavento, Raffaele; Bonderman, Diana; Yuan, Jason.

In: European Respiratory Journal, Vol. 41, No. 2, 01.02.2013, p. 462-468.

Research output: Contribution to journalArticle

Lang, Irene M. ; Pesavento, Raffaele ; Bonderman, Diana ; Yuan, Jason. / Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension : A current understanding. In: European Respiratory Journal. 2013 ; Vol. 41, No. 2. pp. 462-468.
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