Mitja Jevnikar, Sabina Solinas, Philippe Brenot, Benoit Lechartier, Mithum Kularatne, David Montani, Laurent Savale, Carlos Garcia-Alonso, Olivier Sitbon, Antoine Beurnier, Athénaïs Boucly, Sophie Bulifon, Andrei Seferian, Anne Roche, Olaf Mercier, Gérald Simonneau, Elie Fadel, Marc Humbert, Xavier Jaïs

European Respiratory Journal 2023; DOI: 10.1183/13993003.00517-2023

Extract

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.