30 Advances in medical and interventional treatments for CTEPH

Abstract

Pulmonary endarterectomy is the standard care for patients with chronic thromboembolic pulmonary hypertension (CTEPH), however, about 40% of them are inoperable. Several controlled and uncontrolled trials have shown that the use of pulmonary arterial hypertension (PAH)-specific drugs might be useful in inoperable CTEPH.1 2 Riociguat is currently the only PAH-specific drug also approved for inoperable CTEPH.3 Recently, balloon pulmonary angioplasty (BPA) has emerged as an alternative treatment option for patients with inoperable CTEPH or persistent PH after surgery. Several reports now support the efficacy and safety of BPA. The haemodynamic benefits were summarised in a recent review article, with an overall reduction in mean pulmonary arterial pressure of 12–21 mmHg from baseline, and a mortality rate of 0.0%–3.4% after 2–5 angioplasty sessions.4 Sustained haemodynamic improvements, almost to within the normal range, have been reported up to 3.5 years after BPA.5 Severe and fatal complications, including mostly pulmonary vessel injury, may be minimised with not only accumulation of experience but also refinements in technique. An old approach with targeting only one lobe during each session and full balloon sizing increased the incidence of complications. Approaching with undersized balloon may reduce or prevent vessel injury but is less effective in each individual segment, so several segments and lobes are targeted at one session. BPA has the potential to become a key treatment strategy for patients with inoperable CTEPH. However, the indications and limitations of BPA have not been fully established. An international registry contributed by specialised centres is needed for further investigations. References Jais X, D’Armini AM, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, Hoeper MM, Lang IM, Mayer E, Pepke-Zaba J, Perchenet L, Morganti A, Simonneau G, Rubin LJ. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008;52:2127–2134. Ghofrani HA, Simonneau G, D’Armini AM, Fedullo P, Howard LS, Jais X, Jenkins DP, Jing ZC, Madani MM, Martin N, Mayer E, Papadakis K, Richard D, Kim NH. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med 2017;5:785–794. Ghofrani HA, D’Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013;369:319–329. Satoh T, Kataoka M, Inami T, Ishiguro H, Yanagisawa R, Shimura N, Shigeta Y, Yoshino H. Endovascular treatment for chronic pulmonary hypertension: a focus on angioplasty for chronic thromboembolic pulmonary hypertension. Expert Rev Cardiovasc Ther 2016;14:1089–1094. Inami T, Kataoka M, Yanagisawa R, Ishiguro H, Shimura N, Fukuda K, Yoshino H, Satoh T. Long-Term Outcomes After Percutaneous Transluminal Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. Circulation 2016;134:2030–2032.