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Review
. 2023 Feb 16;24(4):3979.
doi: 10.3390/ijms24043979.

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Naoyuki Otani  1 , Ryo Watanabe  2 , Takashi Tomoe  1 , Shigeru Toyoda  2 , Takanori Yasu  1 , Takaaki Nakamoto  1
Affiliations
Review

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Naoyuki Otani et al. Int J Mol Sci. .

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.

Keywords: balloon pulmonary angioplasty; chronic thromboembolic pulmonary hypertension; pulmonary endarterectomy.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results.

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