Chronic thromboembolic pulmonary hypertension

Clin Chest Med. 2013 Dec;34(4):779-97. doi: 10.1016/j.ccm.2013.08.012. Epub 2013 Oct 20.


Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with high mortality and few treatment options. This article reviews the epidemiology of CTEPH and identifies risk factors for its development. The pathobiology and the progression from thromboembolic events to chronically increased right-sided pressures are discussed. The diagnosis and assessment of CTEPH requires several modalities and the role of these is detailed. The pre-operative evaluation assesses peri-operative risk and determines the likelihood of benefit from PTE. Pulmonary thromboendarterectomy (PTE) remains the treatment of choice in appropriate patients. Nonsurgical therapies for CTEPH may provide benefit in patients who cannot be offered surgery.

Keywords: Chronic thromboembolic pulmonary hypertension; Diagnosis; Epidemiology; Percutaneous pulmonary angioplasty; Pulmonary embolus; Pulmonary hypertension; Pulmonary thromboendarterectomy; Therapy.

Publication types

  • Review

MeSH terms

  • Angioplasty
  • Antihypertensive Agents / therapeutic use
  • Chronic Disease
  • Endarterectomy
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / therapy
  • Pulmonary Artery
  • Pulmonary Embolism / complications*
  • Pulmonary Embolism / epidemiology
  • Pulmonary Embolism / therapy
  • Risk Factors
  • United States / epidemiology


  • Antihypertensive Agents