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Effect of macitentan on left ventricular (LV) function in pulmonary arterial hypertension (PAH): results from REPAIR

Session Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure ePosters

Speaker Stephan Rosenkranz

Event : ESC Congress 2020

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Pulmonary Hypertension
  • Session type : ePosters

Authors : S Rosenkranz (Cologne,DE), R Channick (Los Angeles,US), E Cottreel (Allschwil,CH), N Galie (Bologna,IT), D G Kiely (Sheffield,GB), J T Marcus (Amsterdam,NL), A J Swift (Sheffield,GB), A Tawakol (Boston,US), A Torbicki (Otwock,PL), A Vonk Noordegraaf (Amsterdam,NL), G Wetherill (Allschwil,CH), A Peacock (Glasgow,GB)

S Rosenkranz1 , R Channick2 , E Cottreel3 , N Galie4 , D G Kiely5 , J T Marcus6 , A J Swift7 , A Tawakol8 , A Torbicki9 , A Vonk Noordegraaf6 , G Wetherill3 , A Peacock10 , 1Heart Center at the University of Cologne, and Cologne Cardiovascular Research Center (CCRC) - Cologne - Germany , 2David Geffen School of Medicine at UCLA - Los Angeles - United States of America , 3Actelion Pharmaceuticals Ltd - Allschwil - Switzerland , 4Department of Experimental, Diagnostic and Specialty Medicine – DIMES, University of Bologna - Bologna - Italy , 5Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital - Sheffield - United Kingdom of Great Britain & Northern Ireland , 6Amsterdam UMC, Vrije Universiteit Amsterdam - Amsterdam - Netherlands (The) , 7Department of Infection, Immunity & Cardiovascular Disease, University of Sheffield - Sheffield - United Kingdom of Great Britain & Northern Ireland , 8Massachusetts General Hospital - Harvard Medical School - Boston - United States of America , 9Department of Pulmonary Circulation CMKP, European Health Center - Otwock - Poland , 10Scottish Pulmonary Vascular Unit - Glasgow - United Kingdom of Great Britain & Northern Ireland ,

Pulmonary Hypertension

Introduction: PAH impacts right ventricular (RV) structure and function but also leads to changes in the LV due to RV/LV interaction and underfilling. REPAIR, the first PAH study to use a primary endpoint assessed by cardiac MRI (cMRI), reported that RV stroke volume (RVSV) increased by 12 mL and pulmonary vascular resistance (PVR) decreased by 38% from baseline (BL) to Week 26 with macitentan. 

Purpose : To assess the effect of macitentan on LV function in patients with PAH.

Methods: REPAIR (NCT02310672) was a 52-week, multicentre, open-label, single-arm, phase 4 study assessing the effect of macitentan primarily on RV structure and function, determined by cMRI and right heart catheterisation. Macitentan 10 mg was initiated in treatment-naïve patients, in patients receiving stable background phosphodiesterase type-5 inhibitor (PDE5i) at BL, or in initial combination with PDE5i. Exploratory LV endpoints were assessed by cMRI at Weeks 26 and 52. Safety was assessed up to end of study treatment +30 days in all patients who received =1 dose of macitentan (N=87). Patients with BL and Week 26 assessments for both PVR and RVSV were included in the modified Full Analysis Set (mFAS; N=71).

Results: In the mFAS, 57 (80%) patients were female. At BL, median age was 45 years; median (Q1, Q3) six-minute walk distance was 395 (323, 483) m; 48%/51% of patients were WHO functional class II/III; 59% had idiopathic PAH. Compared to BL, at Weeks 26 and 52 there were significant changes in LV cMRI parameters (table). The most common AEs were peripheral oedema (22%), headache (21%) and dizziness (14%).

Conclusions: Macitentan led to improvements in LV mass, volume and function, including clinically-relevant increases in LV stroke volume, at both 26 and 52 weeks in patients with PAH. Safety was consistent with other macitentan clinical trial data.



Mean (SD)

Model-adjusted change from baseline to Week 26

(95% CL)


Model-adjusted change from baseline to Week 52

(95% CL)

LV stroke volume* (mL) 67 47.5 (14.0)

13.8 (10.7, 16.9)



13.8 (10.5, 17.0)


LV ejection fraction* (%) 66 56.3 (10.5)

3.6 (1.1, 6.1)


4.5 (2.0, 7.0)


LV end-diastolic volume (EDV) (mL)* 70 87.2 (29.1)

17.4 (12.4, 22.5)


17.0 (12.7, 21.4)


LV end-systolic volume (ESV) (mL)* 70 32.2 (16.1)

1.7 (-1.0, 4.4)


3.1 (0.6, 5.6)


LV mass (g)* 70 103.4 (23.7)

3.8 (1.4, 6.2)


4.0 (1.1, 7.0)


(RVEDV/LVEDV)** 70 1.8 (0.7)

0.78 (0.76, 0.83)

21% decrease


0.80 (0.77, 0.84)

20% decrease


(RVESV/LVESV)** 70 3.2 (1.6)

0.78 (0.73, 0.83)

22% decrease


0.73 (0.67, 0.80)

27% decrease


#For patients included in the Week 26 analysis.Determined by aortic flow. *From ANCOVA model on parameter change from baseline with a factor for PAH treatment strategy and with the parameter value at baseline as a covariate. **From ANCOVA model on log-transformed ratio of baseline values with a covariate for log-transformed baseline ratios of RVEDV/LVEDV and RVESV/LVESV, respectively. Geometric means ratio to baseline. ANCOVA: analysis of covariance; CL: confidence limit.

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