REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension

Summary

The study evaluates the effect of macitentan on right ventricular and hemodynamic properties in patients with symptomatic pulmonary arterial hypertension. Patients are treated with macitentan for 1 year. Patients undergo right heart catheterization (RHC) at baseline and Week 26. They also undergo cardiac magnetic resonance imaging (MRI) at baseline, Week 26 and Week 52. Safety is monitored throughout the study. The study has three stub-studies. Each patient can participate in no sub-study or in one sub-study. The sub-studies are: (1) metabolism sub-study (with PET-MR scans); (2) biopsy sub-study (biopsies taken during the RHC); (3) Echo sub-study.

Timeline

Start

2015-06-01

Primary completion

2019-09-10

Completion

2019-09-10

Publications

• Kiely DG, Channick R, Flores D, Galie N, MacDonald G, Marcus JT, Mitchell L, Peacock A, Rosenkranz S, Tawakol A, Torbicki A, Vonk Noordegraaf A, Swift AJ. Comparison of cardiac magnetic resonance imaging, functional and haemodynamic variables in pulmonary arterial hypertension: insights from REPAIR. ERJ Open Res. 2024 Feb 12;10(1):00547-2023. doi: 10.1183/23120541.00547-2023. eCollection 2024 Jan.
• Torbicki A, Channick R, Galie N, Kiely DG, Moceri P, Peacock A, Swift AJ, Tawakol A, Vonk Noordegraaf A, Flores D, Martin N, Rosenkranz S. Effect of Macitentan in Pulmonary Arterial Hypertension and the Relationship Between Echocardiography and cMRI Variables: REPAIR Echocardiography Sub-study Results. Cardiol Ther. 2024 Mar;13(1):173-190. doi: 10.1007/s40119-023-00345-2. Epub 2024 Jan 28.
• Vonk Noordegraaf A, Channick R, Cottreel E, Kiely DG, Marcus JT, Martin N, Moiseeva O, Peacock A, Swift AJ, Tawakol A, Torbicki A, Rosenkranz S, Galie N. The REPAIR Study: Effects of Macitentan on RV Structure and Function in Pulmonary Arterial Hypertension. JACC Cardiovasc Imaging. 2022 Feb;15(2):240-253. doi: 10.1016/j.jcmg.2021.07.027. Epub 2021 Nov 17.

Drugs

Indications