Could We Maintain the Initial Efficacy of Triple Sequential Combination Therapies with Selexipag Against Progressive Deterioration Risk in Patients with Pulmonary Arterial Hypertension: Insights from a Single-Center Study?


Tokgöz H. C., Kaymaz C., Kültür Say B., Tanyeri S., Buluş Ç., Sırma D., ...Daha Fazla

Anatolian Journal of Cardiology, cilt.30, sa.4, ss.248-258, 2026 (SCI-Expanded, Scopus, TRDizin)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 30 Sayı: 4
  • Basım Tarihi: 2026
  • Doi Numarası: 10.14744/anatoljcardiol.2025.5976
  • Dergi Adı: Anatolian Journal of Cardiology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CINAHL, EMBASE, MEDLINE, Directory of Open Access Journals, TR DİZİN (ULAKBİM), Academic Search Ultimate (EBSCO), Biomedical Reference Collection: Corporate Edition (EBSCO), Health Research Premium Collection (ProQuest)
  • Sayfa Sayıları: ss.248-258
  • Anahtar Kelimeler: EUPHRATES, pulmonary arterial hypertension, Selexipag
  • Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

Background: This study assessed the efficacy and tolerability of the oral prostacyclin receptor agonist selexipag as part of sequential triple combination therapy in patients with pulmonary arterial hypertension (PAH). Methods: The study retrospectively analyzed 127 of 1160 PAH patients from a single-center registry who received sequential triple therapy including selexipag. Clinical, echocardiographic, and hemodynamic variables and multiparametric risk scores (MRS) were evaluated to assess changes in risk and outcomes. Results: The mean age was 43.2 ± 16.4 years, and 84.3 were female. Prior to selexipag initiation, Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension 2.0 risk strata were: 15 first, 31.5 second, 44.1 third, and 9.4 fourth; European Society of Cardiology/European Respiratory Society low-, intermediate-, and high-risk rates were 20.5, 61.4, and 18.1, respectively. Mean REVEAL Lite 2.0 score was 6.3 ± 2.7. Maximal selexipag dosing reached 1600 µg BID in 18.1 of patients, while 64.6 remained at ≤ 1000 µg BID. Patients were grouped into low-, intermediate-, and high-dose cohorts. Median follow-up was 727.5 days (interquartile range (IQR) 224-985). Selexipag was discontinued in 15 of patients. Across dosing cohorts, initial improvements in functional class, 6-minute walk distance, right ventricular and pulmonary echocardiographic parameters, and MRSs during the first year attenuated thereafter, except for N-terminal pro-brain natriuretic peptide and Tricuspid annular plane systolic excursion/pulmonary arterial systolic pressure ratio. Lower baseline REVEAL Lite 2.0 score predicted low-risk status at final assessment (P = .017). Three-year survival was 72.5, 85.7, and 75.1 in low-, medium-, and high-dose cohorts (P > .05). Mortality was independently predicted by baseline Swedish PAH Registry, REVEAL 2.0, REVEAL Lite 2.0, and REVEAL Echo scores. Conclusion: Earlier escalation to triple therapy with selexipag may improve outcomes. Baseline risk—but not achieved selexipag dose—was associated with survival. A possible decline in treatment effect after 1 year warrants further investigation.