NPPB and Eisenmenger syndrome: The multicenter randomized controlled trial phase III Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity study revealed that NT-proBNP levels and PVR decreased in patients with Eisenmenger syndrome who received macitentan compared with those who received placebo, although it did not show superiority over placebo on the primary endpoint of change from baseline to week 16 in the 6-min walk test (Gatzoulis et al., 2019).