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Initial dual oral combination therapy in inoperable chronic thromboembolic pulmonary hypertension (CTEPH)

There is no data on initial dual oral combination therapy in patients with inoperable CTEPH. The aim of our study was to evaluate the efficacy and safety of different regimen of dual combination of oral PAH drugs initiated in this population. A retrospective analysis was carried out between 2007 and 2017 in 139 consecutive patients with inoperable CTEPH initiated with a combination of endothelin-receptor antagonists (ERAs) with riociguat (RIO) or PDE-5 inhibitor. Results: Mean age was 63±13 years (48% males). ERAs were combined with sildenafil (SIL, n=72), tadalafil (TAD, n=40) or RIO (n=27). In the 117 patients re-evaluated after 4 months (median), there were significant improvements in functional class (class I-II 17% to 39%, p<0.001), 6-min walk distance (307±116 to 346±126 m, p<0.001), mean PAP (52±10 to 45±10 mmHg, p<0.001) and pulmonary vascular resistance (PVR) (13.2±4.6 to 7.8±3.3 WU, p<0.001). Dual combination therapy was well tolerated in all patients. The combination of ERAs with RIO or TAD led to greater reduction in PVR than combination of SIL with an ERA (Table).
 Conclusion: Initial dual oral combination therapy with ERA and drugs targeting NO pathway improves clinical status, exercise capacity and haemodynamics in patients with inoperable CTEPH, similar to that observed in PAH. The combination of ERAs with RIO or TAD was associated with a significantly greater reduction in PVR compared with SIL and an ERA.