Sequential multimodal therapy in chronic thromboembolic pulmonary hypertension (CTEPH) with mixed anatomical lesions: a proof of concept.

Three treatment options have been successfully developed for the management of chronic thromboembolic pulmonary hypertension (CTEPH): pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and pulmonary arterial hypertension (PAH)-targeted medical therapy [1–3]. Management of CTEPH has greatly evolved in the last decade with the possibility to combine these therapeutic options [4, 5]. PEA is the recommended first-line treatment for operable patients [1–3]. However, despite continued improvement in the surgical technique, PEA remains a challenging surgery. This is particularly the case in patients with elevated pulmonary vascular resistance (PVR) as several studies have previously shown that high preoperative PVR was associated with an increase in early post-operative mortality [6–10]. Thus, Tromeur et al . have shown a markedly higher mortality in operated patients with pre-operative PVR of >800 dyn·s·cm−5 [10]. Additionally, some patients may have mixed anatomical lesions where surgically accessible lesions are present in one lung and surgically inaccessible lesions in the contralateral lung. We hypothesized that these patients may be suitable candidates for a sequential multimodal therapy consisting at first of PAH-targeted medical therapy to treat microvasculopathy, then BPA for surgically inaccessible lesions in one lung, and lastly PEA for the surgically accessible lesions in the contralateral lung. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of Interest: Philippe Brenot reports lecture honoraria from MSD, outside the submitted work. Conflict of Interest: David Montani reports grants from Acceleron, Janssen, MSD; consulting fees from Acceleron; lecture honoraria from Bayer, Janssen, MSD; outside the submitted work. Conflict of Interest: Laurent Savale reports grants from Acceleron, Janssen, MSD; lecture honoraria from Janssen, MSD; travel support from Janssen; outside the submitted work. Conflict of Interest: Olivier Sitbon reports grants from Acceleron, Janssen, MSD, GSK; consulting fees from Gossamer Bio, Janssen, MSD; lecture honoraria from AOP Orphan, Janssen, Ferrer, MSD; travel support from Janssen; advisory board participation from Acceleron, Janssen, MSD; outside the submitted work. Conflict of Interest: Athénaïs Boucly reports grants from Acceleron, Janssen and MSD, payment or honoraria for lectures, manuscript writing or educational events from Janssen, Merck and AOP Orphan, and support for attending meetings and/or travel from Janssen. Conflict of Interest: Elie Fadel reports lecture honoraria from MSD, outside the submitted work. Conflict of Interest: Gérald Simonneau reports grants from Acceleron, Janssen, MSD; consulting fees and advisory board participation with Acceleron, Janssen, MSD, Bayer; lecture honoraria from Bayer, Janssen, MSD; travel support from Janssen; outside the submitted work. Conflict of Interest:Marc Humbert reports grants and consulting fees from Acceleron, Janssen, MSD; lecture honoraria from Janssen, MSD; advisory board participation with Acceleron, Janssen, MSD, United Therapeutics; and has an interest in Investissement d'Avenir program managed by the French National Research Agency under the grant contract ANR-18-RHUS-0006 (DESTINATION 2024); outside the submitted work. Conflict of Interest: Xavier Jaïs reports grants from Acceleron, Janssen, MSD; lecture honoraria from Janssen, MSD; travel support from Janssen; outside the submitted work. Conflict of Interest: All other authors have nothing to disclose.