Medical and interventional therapies for inoperable CTEPH: a necessary combination?

Surgical pulmonary endarterectomy (PEA) is a potential cure for chronic thromboembolic pulmonary hypertension (CTEPH), a rare pulmonary vascular disease, but the procedure requires a high level of skill and roughly half of patients remain surgically untreatable. In The Lancet Respiratory Medicine, the RACE study by Xavier Jaïs and colleagues 1 Jaïs X Brenot P Bouvaist H et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med. 2022; (published online Aug 1.)https://doi.org/10.1016/S2213-2600(22)00214-4 Google Scholar and the MR BPA study by Takashi Kawakami and colleagues 2 Kawakami T Matsubara H Shinke T et al. Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension (MR BPA): an open-label, randomised controlled trial. Lancet Respir Med. 2022; (published online Aug 1.)https://doi.org/10.1016/S2213-2600(22)00171-0 Google Scholar compare the efficacy and safety of balloon pulmonary angioplasty (BPA) with those of the soluble guanylate cyclase stimulator riociguat in patients with inoperable CTEPH. What can we learn from these studies, which have some similarities as well as important differences? Key questions include whether medical therapy with riociguat is still needed given the hitherto reported efficacy of BPA, albeit in uncontrolled studies, or whether the risk–benefit ratio of medical therapy outweighs the therapeutic potential of BPA. And, importantly, whether these treatments could be combined to increase efficacy or improve safety. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up studyAt week 26, pulmonary vascular resistance reduction was more pronounced with BPA than with riociguat, but treatment-related serious adverse events were more common with BPA. The finding of fewer BPA-related serious adverse events among patients who were pretreated with riociguat in the follow-up study compared with those who received BPA as first-line treatment points to the potential benefits of a multimodality approach to treatment in patients with inoperable CTEPH. Further studies are needed to explore the effects of sequential treatment combining one or two medications and BPA in patients with inoperable CTEPH. Full-Text PDF Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension (MR BPA): an open-label, randomised controlled trialCompared with riociguat, BPA was associated with a greater improvement in mean pulmonary arterial pressure in patients with inoperable CTEPH at 12 months, although procedure-related complications were reported. These findings support BPA as a reasonable option for inoperable CTEPH in centres with experienced BPA operators, with attention to procedure-related complications. Full-Text PDF Standards for assessing and reporting adverse eventsWe thank Hossein-Ardeschir Ghofrani and Nick Kim1 for their positive remarks on the Multicentre Randomised controlled trial based on Balloon Pulmonary Angioplasty (MR BPA) study.2 We agree that our results and those of the RACE trial3 point to a combined role for medical therapy and BPA in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, in interpreting the findings of the two trials, Ghofrani and Kim1 noted that the definitions of adverse and serious adverse events were markedly different in RACE3 and MR BPA;2 we therefore believe that an explanation of the definitions used in our study—and the standards used in Japan and globally—would be useful. Full-Text PDF