Molecular Biology of Chronic Thromboembolic Pulmonary Hypertension

Richard S. Sacks, Carmelle V. Remillard, Negin Agange, William R. Auger, Patricia A. Thistlethwaite, Jason X.J. Yuan

Research output: Contribution to journalArticlepeer-review

25 Scopus citations


Recent efforts have seen major advances in elucidating the mechanisms underlying pulmonary arterial hypertension. However, chronic thromboembolic pulmonary hypertension (CTEPH) often has been excluded from these studies. Consequently, whereas the clinical, radiographic, and hemodynamic characteristics of CTEPH have been well described, there remains a deficit in our understanding of the cellular, molecular, and genetic mechanisms underlying CTEPH. Furthermore, although prior venous thromboembolism may act as the inciting event, it is still unclear what predisposes some patients to develop CTEPH. CTEPH has two major pathogenic components. The first is the primary obstruction of central pulmonary arteries by accumulation of thrombotic material. The second is characterized by severe pulmonary vascular remodeling, similar to that seen in idiopathic pulmonary arterial hypertension. Other articles in this series describe the pathological, surgical, and therapeutic aspects of CTEPH. Here, we review the potential molecular and cellular mechanisms that may contribute to the pathogenesis of CTEPH.

Original languageEnglish (US)
Pages (from-to)265-276
Number of pages12
JournalSeminars in thoracic and cardiovascular surgery
Issue number3
StatePublished - 2006
Externally publishedYes


  • pulmonary embolism
  • pulmonary hypertension
  • remodeling
  • thrombus

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine


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