MR-augmented cardio pulmonary exercise testing: an integrated approach to assessment of children with pulmonary hypertension

Background Pulmonary arterial hypertension (PAH) is a life limiting disorder resulting in progressive right heart (RV) failure. Cardiac MRI is the reference standard for RV assessment in PH and has known prognostic value in children. Symptoms of PAH are exacerbated or become apparent with exercise and cardiopulmonary exercise testing (CPET) is also prognostic in PAH. We have recently demonstrated the feasibility of MR augmented CPET (MR-CPET) in healthy adults. Using the Fick principle MR-CPET allows calculation of tissue oxygen extraction ΔcO2. A combined approach using MR-CPET could provide a more sensitive method of assessing disease severity, the causes of exercise limitation and response to treatment. In this study we assessed the utility of MR-CPET in children with PAH.