Effect of Macitentan in Pulmonary Arterial Hypertension and the Relationship Between Echocardiography and cMRI Variables: REPAIR Echocardiography Sub-study Results.

Jan 29, 2024

Contributors: Adam Torbicki,Richard Channick,Nazzareno Galiè,David G Kiely,Pamela Moceri,Andrew Peacock,Andrew J Swift,Ahmed Tawakol,Anton Vonk Noordegraaf,Dayana Flores,Nicolas Martin,Stephan Rosenkranz

The aim of this sub-study was to evaluate the relationship between echocardiography (echo) and cardiac magnetic resonance imaging (cMRI) variables and to utilize echo to assess the effect of macitentan on right ventricle (RV) structure and function.
REPAIR (NCT02310672) was a prospective, multicenter, single-arm, open-label, 52-week, phase 4 study in pulmonary arterial hypertension (PAH) patients, which investigated the effect of macitentan 10 mg as monotherapy, or in combination with a phosphodiesterase 5 inhibitor, on RV structure, function, and hemodynamics using cMRI and right heart catheterization. In this sub-study, patients were also assessed by echo at screening and at weeks 26 and/or 52. Post hoc correlation analyses between echo and cMRI variables were performed using Pearson’s correlation coefficient, Spearman’s correlation coefficient, and Bland-Altman analyses.
The Echo sub-study included 45 patients. Improvements in echo-assessed RV stroke volume (RVSV), left ventricular SV (LVSV), LV end-diastolic volume (LVEDV), RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), and in 2D global longitudinal RV strain (2D GLRVS) were observed at weeks 26 and 52 compared to baseline. There was a strong correlation between echo (LVSV, 2D GLRVS, and LVEDV) and cMRI variables, with a moderate correlation for RVSV. Bland-Altman analyses showed a good agreement for LVSV measured by echo versus cMRI, whereas an overestimation in echo-assessed RVSV was observed compared to cMRI (bias of – 15 mL). Hemodynamic and functional variables, as well as safety, were comparable between the Echo sub-study and REPAIR.
A good relationship between relevant echo and cMRI parameters was shown. Improvements in RV structure and function with macitentan treatment was observed by echo, consistent with results observed by cMRI in the primary analysis of the REPAIR study. Echo is a valuable complementary method to cMRI, with the potential to non-invasively monitor treatment response at follow-up.
REPAIR NCT02310672.

© 2024. The Author(s).

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ABOUT THE CONTRIBUTORS

Adam Torbicki,Richard Channick,Nazzareno Galiè,David G Kiely,Pamela Moceri,Andrew Peacock,Andrew J Swift,Ahmed Tawakol,Anton Vonk Noordegraaf,Dayana Flores,Nicolas Martin,Stephan Rosenkranz
Adam Torbicki

Department of Pulmonary Circulation, Thromboembolic Disease and Cardiology, Centre for Postgraduate Medical Education ECZ-Otwock, ERN-LUNG Member, F. Chopin Hospital European Health Centre, ul. Borowa 14/18, 05-400, Otwock, Poland. atorbicki@gmail.com.

Richard Channick

David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.

Nazzareno Galiè

Cardiology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna and Dipartimento DIMES, Università di Bologna, Bologna, Italy.

David G Kiely

Sheffield Pulmonary Vascular Disease Unit, NIHR Biomedical Research Centre Sheffield and University of Sheffield, Sheffield, UK.

Pamela Moceri

Cardiology Department, UR2CA, Pasteur University Hospital, Côte-d’Azur University, Nice, France.

Andrew Peacock

Scottish Pulmonary Vascular Unit, Glasgow, UK.

Andrew J Swift

Department of Infection, Immunity & Cardiovascular Disease, National Institute for Health and Care Research Sheffield Biomedical Research Centre, University of Sheffield, Sheffield, UK.

Ahmed Tawakol

Massachusetts General Hospital and Harvard Medical School, Boston, USA.

Anton Vonk Noordegraaf

Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.

Dayana Flores

Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson, Global Medical Affairs, Allschwil, Switzerland.

Nicolas Martin

Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson, Statistical Decision Science, Allschwil, Switzerland.

Stephan Rosenkranz

Department of Cardiology, Heart Center, University Hospital Cologne, and Cologne Cardiovascular Research Center (CCRC), University of Cologne, Cologne, Germany.