Pulmonary arterial hypertension (PAH) studies, both complete and ongoing, utilising cardiovascular magnetic resonance (CMR) endpoints
| Study | Type of study | Patients n | Aim | CMR endpoints | Key results summary |
| Van Wolferen [10] (2007) | Prospective observational study | 64 | Investigate the relationship between right ventricular structure, function and survival in IPAH | CI RVSVI RVMI LVMI RVWT LVWT RVEF LVEF RVEDVI LVEDVI | The 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 study | 110 | Examine the relationship between changes in PVR, RVEF and survival in patients with PAH under PAH-targeted therapies | RVEDVI RVESVI RVEF LVEDVI LVESVI LVEF RVSVI | Loss of right ventricular function is associated with a poor outcome, irrespective of any changes in PVR |
| Wilkins [103] (2005), SERAPH | Randomised controlled trial | 26 | Compare the effect of adding sildenafil, a PDE-5i, to bosentan, an ERA (and the conventional treatment) in PAH | RVM | RVM decreased with addition of sildenafil to bosentan (mean −8.8 g versus −3 g; p=0.142) |
| Wilkins [104] (2010), SiPHT | Randomised controlled trial | 42 | Assess the therapeutic value of simvastatin in patients with PAH | RVM | Simvastatin 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 study | 11 | Assess changes in main pulmonary artery flow, as well as RVM and volumes, in patients with PH during epoprostenol therapy | RVM RVEDV | No significant changes noted in either parameter |
| Van Wolferen [105] (2006) | Prospective observational study | 15 | Investigate whether the addition of sildenafil reverses right ventricular remodelling and improvement in right ventricular function in PAH treated with bosentan | RVM RVEF RVEDV | Addition 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 study | 16 | Investigate changes in CMR measurements in PAH treated with bosentan and compare cardiac MRI results with other known markers of prognosis, including RHC and 6 MWT | RVEF RVEDV RVSV | No significant changes noted with bosentan therapy |
| Peacock [91] (2014), EURO-MR | Prospective observational study | 91 | Assessment of right ventricular function using CMR to determine response to therapy (ERA or PDE-5i) and to monitor disease progression in PH | RVEDVI RVSVI RVEF RVMI | Significant improvement noted in RVEF (p<0.001) and RVSVI (p<0.0001) |
| Van de Veerdonk [106] (2017) | Retrospective study | 80 | Assess the impact of upfront combination therapy (ERA+PDE-5i) on right ventricular volume compared with monotherapy (ERA or PDE-5i) in PAH | RVEDV RVESV RVM RVEF Relative RVWT | Right 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–O | Prospective, multicentre study | 24 | Evaluate the effect of upfront combination therapy with ambrisentan and tadalafil on left and right ventricular function in patients with SSc-PAH | RVM | Significantly greater reductions in RVM noted with combination therapy (28.0 g versus 32.5 g; p<0.05) |
| REPAIR (Clinicaltrials.gov NCT02310672) | Prospective, multicentre, single-arm study | In progress | Assess the effect of macitentan on right ventricular and haemodynamic properties (at baseline and 26 weeks) in patients with symptomatic PAH | Changes in RVSV Ventriculo-arterial coupling measured by CMR | In progress |
| REPLACE (Clinicaltrials.gov NCT02829034) | Multicentre, randomised controlled trial versus placebo | In progress | Assess the effectiveness of riociguat as a replacement for PDE-5i therapy in PAH | Change in RVEF measured by CMR | In 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.