Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Rabea Asleh, Offer Amir, Neville Berkman

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review


Chronic thromboembolic pulmonary hypertension (CTEPH) is a relatively rare consequence of recurrent acute pulmonary embolic events. Screening for CTEPH post-acute PE is recommended only in patients with persistent symptoms. The disease is characterized by a combination of organized fibrotic clots in the larger proximal pulmonary arteries and a small vessel vasculopathy analogous to that seen in idiopathic PAH. The clinical presentation of CTEPH is one of progressive dyspnea followed by development of right heart failure. Diagnosis requires right heart catheterization together with an abnormal ventilation-perfusion lung scintigraphy scan. Lifelong anticoagulation is indicated. Treatment of choice in appropriate cases is surgical pulmonary thromboendarterectomy (PEA). Balloon pulmonary angioplasty (BPA) as well as medical therapy improve hemodynamics and functional class in patients not suitable for surgical intervention. There are ongoing studies aimed at identifying which treatment option or combination of treatments, are best suited for which patients.

Original languageEnglish
Title of host publicationPulmonary Embolism
PublisherSpringer International Publishing
Number of pages13
ISBN (Electronic)9783030870904
ISBN (Print)9783030870898
StatePublished - 1 Jan 2021
Externally publishedYes

Bibliographical note

Publisher Copyright:
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022.


  • Balloon pulmonary angioplasty (BPA)
  • CT-pulmonary angiography (CTPA)
  • Chronic thrombo-embolic pulmonary hypertension
  • Pulmonary thromboendarterectomy (PEA)
  • Riociguat


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