FIGURE 1:

Study flow diagram. ^aOne participant withdrew from the study because of an AE; however, this participant’s AE was actually a protocol-defined stopping criterion and is presented here in the stopping criteria category rather than the AE category. ^bCompleted all study visits or abbreviated schedule if study medication was discontinued. ^cFifteen participants discontinued study medication because of AEs; however, five of these participants had AEs that were actually protocol-defined stopping criteria and are presented here in the stopping criteria category rather than the AE category. ^dOne participant discontinued study medication because of an AE; however, this participant’s AE was actually a protocol-defined stopping criterion and is presented here in the stopping criteria category rather than the AE category. ^eProtocol-defined reasons for discontinuation of study medication included hemoglobin <7.5 g/dL, renal transplant, increased sPAP of ≥20 mmHg, drop of LVEF ≥10% from baseline and <50% and blood transfusion. The complete list of stopping criteria is included in Supplementary data, Item S1. IP: investigational product; PI: principal investigator.