American Society of Hirudotherapy

Chronic thromboembolic pulmonary hypertension

Review published in The Lancet. Respiratory medicine (2014)

Last Updated: November 1, 2026Reviewed by: ASH Editorial Board
Research article — evidence reviewArticle reference
Evidence: Narrative reviewClinical LiteratureDrug DevelopmentHoeper MM et al. · The Lancet. Respiratory medicine, 2014

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening complication of acute pulmonary embolism. CTEPH results from persistent obstruction of pulmonary arteries and progressive vascular remodelling. Not all patients presenting with CTEPH have a history of clinically overt pulmonary embolism. The diagnostic work-up to detect or rule out CTEPH should include ventilation-perfusion scintigraphy, which has high sensitivity and a negative predictive value of nearly 100%. CT angiography usually reveals typical features of CTEPH, including mosaic perfusion, part or complete occlusion of pulmonary arteries, and intraluminal bands and webs. Patients with suspected CTEPH should be referred to a specialist centre for right-heart catheterisation and pulmonary angiography. Surgical pulmonary endarterectomy remains the treatment of choice for CTEPH and is associated with excellent long-term results and a high probability of cure. For patients with inoperable CTEPH, various medical and interventional therapies are being developed.

Abstract sourced from PubMed (NCBI) for the cited record. See the original publication for the authoritative version.

Publication typeJournal ArticleReview
Indexed MeSH termsAngioplasty, BalloonBosentanChronic DiseaseEndarterectomyEndothelin Receptor AntagonistsEnzyme ActivatorsHumansHypertension, PulmonaryPhosphodiesterase 5 InhibitorsPiperazinesPulmonary EmbolismPurinesPyrazolesPyrimidinesSildenafil CitrateSulfonamidesThromboembolism

Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening complication of acute pulmonary embolism. CTEPH results from persistent obstruction of pulmonary arteries and progressive vascular remodelling. Not all patients presenting with CTEPH have a history of clinically overt pulmonary embolism.

Citation

Reference (Vancouver style)

Hoeper MM, Madani MM, Nakanishi N, Meyer B, Cebotari S, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Lancet Respir Med. 2014;2(7):573-582. doi:10.1016/S2213-2600(14)70089-X. PMID: 24898750

Bibliographic details (authors, volume, pages, DOI) are taken from the PubMed record.

DOIPubMed

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