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Lung. 2019 Jun;197(3):353-360. doi: 10.1007/s00408-019-00222-7. Epub 2019 Apr 8.

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.

Author information

1
Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.
2
Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland. silvia.ulrich@usz.ch.
3
Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland. silvia.ulrich@usz.ch.

Abstract

BACKGROUND:

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.

METHODS:

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).

RESULTS:

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.

CONCLUSIONS:

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.

KEYWORDS:

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

PMID:
30963265
DOI:
10.1007/s00408-019-00222-7

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