Journal article

Publication year: 2021

Journal: Respiratory Medicine

Volume number: 177

ISSN: 0954-6111

eISSN: 1532-3064

Open access status: Hybrid

DOI Link: https://doi.org/10.1016/j.rmed.2020.106293

Publisher: Elsevier

Abstract: 
Chronic thmmboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the pulmonary vasculature, leading to increased pulmonary vascular resistance and ultimately right ventricular failure, the leading cause of death in non-operated patients. This article reviews the current management of CTEPH. The standard of care in CTEPH is pulmonary endarterectomy (PEA). However, up to 40% of patients with CTEPH are ineligible for PEA, and up to 51% develop persistent/recurrent PH after PEA. Riociguat is currently the only medical therapy licensed for treatment of inoperable or persistent/recurrent CTEPH after PEA based on the results of the Phase III CHEST-1 study. Studies of balloon pulmonary angioplasty (BPA) have shown benefits in patients with inoperable or persistent/recurrent CTEPH after PEA; however, data are lacking from large, prospective, controlled studies. Studies of macitentan in patients with inoperable CTEPH and treprostinil in patients with inoperable or persistent/recurrent CTEPH showed positive results. Combination therapy is under evaluation in CTEPH, and long-term data are not available. In the future, CTEPH may be managed by PEA, medical therapy or BPA - alone or in combination, according to individual patient needs. Patients should be referred to experienced centers capable of assessing and delivering all options.

Harvard Citation style: Ghofrani, H., D'Armini, A., Kim, N., Mayer, E. and Simonneau, G. (2021) Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension, Respiratory Medicine, 177, Article 106293. https://doi.org/10.1016/j.rmed.2020.106293

APA Citation style: Ghofrani, H., D'Armini, A., Kim, N., Mayer, E., & Simonneau, G. (2021). Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension. Respiratory Medicine. 177, Article 106293. https://doi.org/10.1016/j.rmed.2020.106293