, Volume 197, Issue 3, pp 353–360 | Cite as

Real-Life Experience with Selexipag as an Add-On Therapy to Oral Combination Therapy in Patients with Pulmonary Arterial or Distal Chronic Thromboembolic Pulmonary Hypertension: A Retrospective Analysis

  • Charlotte Berlier
  • Esther I. Schwarz
  • Stéphanie Saxer
  • Mona Lichtblau
  • Silvia UlrichEmail author
Pulmonary Hypertension



Patients with pulmonary arterial hypertension (PAH) and distal chronic thromboembolic pulmonary hypertension (CTEPH) who still reveal risk factors of worse prognosis on double combination therapy may benefit from add-on therapy with the novel oral selective prostacyclin receptor agonist selexipag.


We reviewed all patients with PAH/distal CTEPH in the Zurich cohort who received selexipag as add-on to oral combination therapy and retrieved New York Heart Association (NYHA) functional class, 6-min walk distance (6MWD), NT-pro-BNP, quality of life questionnaires (CAMPHOR and EuroQoL), tricuspid pressure gradient (TPG) by echocardiography and cardiopulmonary exercise test parameters (power output and oxygen uptake).


Twenty-three patients with PAH/CTEPH (20/3), 14 females, median (quartiles) age 56 (46; 66) years received an oral triple therapy containing selexipag at a median dose of 2000 (1600; 3100) mcg during 221 (113; 359) days. The following parameters were stabilized from baseline to last FU: 6MWD (440 (420; 490) to 464 (420; 526) m), NYHA class (three to two), NT-pro-BNP (326 (167; 1725) to 568 (135; 1856)  ng/l), TPG, power output, and oxygen uptake. Quality of life reflected by the CAMPHOR and EuroQoL improved.


Early initiation of triple oral combination therapy including selexipag in PAH/CTEPH with intermediate risk factor profile may help to stabilize functional class, exercise performance, and pulmonary hemodynamics in a real-life setting and potentially improves quality of life. Whether these beneficial effects can be truly attributed to the addition of selexipag should be addressed in future randomized controlled trials.


Pulmonary hypertension Pulmonary arterial hypertension Vasodilator therapy Endothelin receptor antagonist Phosphodiesterase inhibitor Selexipag Chronic thromboembolic pulmonary hypertension 



The study was funded by grants for the Zurich PH cohort from the Zurich Lung.

Compliance with Ethical Standards

Conflict of interest

None of the authors declares any conflict of interest in relation to the present work. SU received grant money from the Swiss National Science Foundation, Zurich Lung, Actelion SA, Bayer SA, Orpha Swiss. SU; EIS, SS, ML and CB received travel support and lecture fees from Actelion SA, Bayer SA, MSD SA and Orpha Swiss.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This retrospective cohort study complied with the ethical laws in Switzerland and all patients have signed informed consent for the Zurich PH cohort study (KEK 2014-0214).


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Charlotte Berlier
    • 1
  • Esther I. Schwarz
    • 1
  • Stéphanie Saxer
    • 1
  • Mona Lichtblau
    • 1
  • Silvia Ulrich
    • 1
    • 2
    Email author
  1. 1.Clinic of PulmonologyUniversity Hospital of ZurichZurichSwitzerland
  2. 2.Center for Integrative Human PhysiologyUniversity of ZurichZurichSwitzerland

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