Science and Research

Right ventricular size and function under riociguat in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (the RIVER study)

BACKGROUND: Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTPEH). The objective of this study was to evaluate right heart size and function assessed by echocardiography during long term treatment with riociguat. METHODS: Patients who started riociguat treatment (1.0-2.5 mg tid) within the trials phase II, PATENT, PATENTplus, EAS, CHEST and continued treatment for 3-12 months were included in this study. Echocardiography was analysed off-line at baseline, after 3, 6 and 12 months by investigators who were blinded to clinical data. Last and baseline observation carried forward method (LOCF, BOCF) were performed as sensitivity analysis. RESULTS: Seventy-one patients (45% PAH, 55% CTEPH; 53.5% female; 60 +/- 13 years, mean pulmonary arterial pressure 46 +/- 10 mmHg, mean PVR 700 +/- 282dynes.sec.cm-5) were included. After 6 months, RA and RV area, RV thickness tricuspid regurgitation velocity showed a significant reduction. After 12 months, patients receiving riociguat therapy showed a significant reduction in right atrial (- 2.6 +/- 4.4 cm2, 95% CI -3.84, - 1.33; p < 0.001, n = 49) and right ventricular (RV) area (- 3.5 +/- 5.2 cm2, 95% CI -5.1, - 1.9; p < 0.001; n = 44), RV thickness (- 0.76 +/- 2.2 mm, 95% CI -1.55, 0.03; n = 32), and a significant increase in TAPSE (2.95 +/- 4.78 mm, 95% CI 1.52, 4.39; n = 45) and RV fractional area change (8.12 +/- 8.87 mm, 95% CI 4.61, 11.62; n = 27). Both LOCF and BOCF showed similar results but lower effect sizes. CONCLUSION: Patients under long-term treatment with riociguat show significantly reduced right heart size and improved RV function in PAH and CTEPH. Further controlled prospective studies are needed to confirm these results.
  • Marra, A. M.
  • Halank, M.
  • Benjamin, N.
  • Bossone, E.
  • Cittadini, A.
  • Eichstaedt, C. A.
  • Egenlauf, B.
  • Harutyunova, S.
  • Fischer, C.
  • Gall, H.
  • Ghofrani, H. A.
  • Hoeper, M. M.
  • Lange, T. J.
  • Olsson, K. M.
  • Klose, H.
  • Grunig, E.

Keywords

  • Aged
  • Chronic Disease
  • Double-Blind Method
  • Enzyme Activators/pharmacology/therapeutic use
  • Female
  • Heart Ventricles/diagnostic imaging/*drug effects
  • Humans
  • Hypertension, Pulmonary/diagnostic imaging/*drug therapy
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulmonary Embolism/diagnostic imaging/*drug therapy/physiopathology
  • Pyrazoles/pharmacology/*therapeutic use
  • Pyrimidines/pharmacology/*therapeutic use
  • Retrospective Studies
  • Ventricular Function, Right/*drug effects/physiology
  • Chronic thromboembolic pulmonary hypertension
  • Echocardiography
  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Right atrial area
  • Right heart size
  • Right ventricular function
  • Riociguat
  • Soluble guanylate cyclase stimulator
Publication details
DOI: 10.1186/s12931-018-0957-y
Journal: Respiratory research
Pages: 258 
Number: 1
Work Type: Original
Location: TLRC, UGMLC
Disease Area: PH
Partner / Member: JLU, Thorax
Access-Number: 30567595
See publication on PubMed

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