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Advances in targeted therapy for chronic thromboembolic pulmonary hypertension

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by unresolved thrombi in the pulmonary arteries and microvasculopathy in nonoccluded areas. If left untreated, progressive pulmonary hypertension will induce right heart failure and, finally, death. Currently, pulmonary endarterectomy (PEA) remains the only method that has the potential to cure CTEPH. Unfortunately, up to 40% of patients are ineligible for this procedure for various reasons. In recent years, refined balloon pulmonary angioplasty (BPA) has become an alternative option for inoperable CTEPH patients, and it may be another curative treatment in the future, particularly in combination with prior PEA. Nevertheless, 23% of patients still suffer from persistent PH after BPA. Given that CTEPH shares many similarities with idiopathic pulmonary arterial hypertension (PAH), targeted drugs developed for PAH are also attractive options for CTEPH, especially for inoperable or persistent/recurrent CTEPH patients. To date, riociguat, macitentan, and subcutaneous treprostinil are the only drugs proven by randomized control trials to be capable of improving the exercise capacity (6-min walking distance) of CTEPH patients. In this review, we summarize the achievements and unresolved problems of PAH-targeted therapy for CTEPH over the last decade.

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Funding

This review article was supported by the National Natural Science Foundation of China (81370326, 81641005), Beijing Municipal Science and Technology Project (Z181100001718200), and National Precision Medical Research Program of China (2016YFC0905602).

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  1. Yi Zhang and Xue Yu contributed equally to this work.

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  1. Center for Pulmonary Vascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167 Beilishi Rd, Xicheng District, Beijing, 100037, China

    Yi Zhang, Xue Yu, Qi Jin, Qin Luo, Zhihui Zhao, Qing Zhao, Lu Yan & Zhihong Liu

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  1. Yi Zhang
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Zhang, Y., Yu, X., Jin, Q. et al. Advances in targeted therapy for chronic thromboembolic pulmonary hypertension. Heart Fail Rev 24, 949–965 (2019). https://doi.org/10.1007/s10741-019-09798-x

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