Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension. (February 2021)

• Record Type: Journal Article
• Authors: Ghofrani, Hossein-Ardeschir; D'Armini, Andrea M.; Kim, Nick H.; Mayer, Eckhard; Simonneau, Gérald
• Is Part Of: Respiratory medicine. Volume 177(2021)
• Publication Date: 2021-02
• Subjects: Riociguat -- CTEPH -- Hypertension -- Pulmonary -- Endarterectomy -- Balloon pulmonary angioplasty; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2020.106293
• Languages: English
• ISSNs: 0954-6111

Abstract

Abstract: Chronic thromboembolic 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. Highlights: PEA is standard of care for operable CTEPH. Many patients are inoperable or have persistent/recurrent CTEPH after PEA. For patients with inoperable CTEPH, BPA and/or medical therapy are recommended. There is increasing evidence for the efficacy of BPA for inoperable CTEPH. Riociguat is the only drug approved for inoperable and persistent/recurrent CTEPH.