Pulmonary arterial hypertension (PAH) studies, both complete and ongoing, utilising cardiovascular magnetic resonance (CMR) endpoints

StudyType of studyPatients nAimCMR endpointsKey results summary
Van Wolferen [10] (2007)Prospective observational study64Investigate the relationship between right ventricular structure, function and survival in IPAHCI RVSVI RVMI LVMI RVWT LVWT RVEF LVEF RVEDVI LVEDVIThe following are strong independent predictors of mortality and treatment failure: large right ventricular volume; low stroke volume; reduced left ventricular volume
Van de Veerdonk [8] (2011)Prospective observational study110Examine the relationship between changes in PVR, RVEF and survival in patients with PAH under PAH-targeted therapiesRVEDVI RVESVI RVEF LVEDVI LVESVI LVEF RVSVILoss of right ventricular function is associated with a poor outcome, irrespective of any changes in PVR
Wilkins [103] (2005), SERAPHRandomised controlled trial26Compare the effect of adding sildenafil, a PDE-5i, to bosentan, an ERA (and the conventional treatment) in PAHRVMRVM decreased with addition of sildenafil to bosentan (mean −8.8 g versus −3 g; p=0.142)
Wilkins [104] (2010), SiPHTRandomised controlled trial42Assess the therapeutic value of simvastatin in patients with PAHRVMSimvastatin added to conventional therapy produced a small and transient early reduction in RVM but this was not sustained over 12 months
Roeleveld [92] (2004)Prospective observational study11Assess changes in main pulmonary artery flow, as well as RVM and volumes, in patients with PH during epoprostenol therapyRVM RVEDVNo significant changes noted in either parameter
Van Wolferen [105] (2006)Prospective observational study15Investigate whether the addition of sildenafil reverses right ventricular remodelling and improvement in right ventricular function in PAH treated with bosentanRVM RVEF RVEDVAddition of Sildenafil to bosentan significantly decreased RVM (p<0.01) and increased RVEF (p<0.05) with no significant change in RVEDV
Chin [11] (2008)Prospective observational study16Investigate changes in CMR measurements in PAH treated with bosentan and compare cardiac MRI results with other known markers of prognosis, including RHC and 6 MWTRVEF RVEDV RVSVNo significant changes noted with bosentan therapy
Peacock [91] (2014), EURO-MRProspective observational study91Assessment of right ventricular function using CMR to determine response to therapy (ERA or PDE-5i) and to monitor disease progression in PHRVEDVI RVSVI RVEF RVMISignificant improvement noted in RVEF (p<0.001) and RVSVI (p<0.0001)
Van de Veerdonk [106] (2017)Retrospective study80Assess the impact of upfront combination therapy (ERA+PDE-5i) on right ventricular volume compared with monotherapy (ERA or PDE-5i) in PAHRVEDV RVESV RVM RVEF Relative RVWTRight ventricular volumes, calculated right ventricular wall stress and RVEF improved after combination therapy (p<0.001) but remained unchanged after monotherapy
Hassoun [107] (2015), ATPAHSS–OProspective, multicentre study24Evaluate the effect of upfront combination therapy with ambrisentan and tadalafil on left and right ventricular function in patients with SSc-PAHRVMSignificantly greater reductions in RVM noted with combination therapy (28.0 g versus 32.5 g; p<0.05)
REPAIR ( NCT02310672)Prospective, multicentre, single-arm studyIn progressAssess the effect of macitentan on right ventricular and haemodynamic properties (at baseline and 26 weeks) in patients with symptomatic PAHChanges in RVSV Ventriculo-arterial coupling measured by CMRIn progress
REPLACE ( NCT02829034)Multicentre, randomised controlled trial versus placeboIn progressAssess the effectiveness of riociguat as a replacement for PDE-5i therapy in PAHChange in RVEF measured by CMRIn progress

IPAH: idiopathic PAH; CI: cardiac index; RVM: right ventricular mass; RVMI: right ventricular mass index; LVMI: left ventricular mass index; RVWT: right ventricular wall thickness; LVWT: left ventricular wall thickness; RVEF: right ventricular ejection fraction; LVEF: left ventricular ejection fraction; RVEDV: right ventricular end diastolic volume; RVEDVI: right ventricular end diastolic volume index; LVEDVI: left ventricular end diastolic volume index; PVR: pulmonary vascular resistance; LVESVI: left ventricular end systolic volume index; RVESV: right ventricular end systolic volume; RVESVI: right ventricular end systolic volume index; RVSV: right ventricular stroke volume; RVSVI: right ventricular stroke volume index; PDE-5i: phosphodiesterase type-5 inhibitor; ERA: endothelin receptor antagonist; MRI: magnetic resonance imaging; RHC: right-heart catheterisation; 6MWT: 6-min walk test; SSc-PAH: systemic sclerosis associated PAH.