case_images: PMC9247454_01
case_images: PMC9247454_02
case_images: Timeline of selexipag initiation at the age of 14-16 years. Day 0 indicates the day on which selexipag was initiated. RVp, right ventricular pressure; LVp, left ventricular pressure; UCG, ultrasoundcardiography.
case_images: fped-10-909595-g001.jpg
case_images: PMC9247454_02_fped-10-909595-g001.jpg
case_images: F1
case_images: After titration of selexipag to 0.8 mg/day, ultrasound cardiography showed that the estimated systolic right ventricular pressure improved to approximately 50% of the systolic left ventricular pressure (Figure 1).
case_images: The drug transition timeline at the age of 17 years. Day 0 indicates the day on which the dose of selexipag was increased to 1.6 mg/day. RVp, right ventricular pressure; LVp, left ventricular pressure; UCG, ultrasoundcardiography.
case_images: fped-10-909595-g002.jpg
case_images: PMC9247454_02_fped-10-909595-g002.jpg
case_images: F2
case_images: At 17 years of age, we increased the dose of selexipag to 2.8 mg/day and decreased the dose of epoprostenol from 31 to 10 ng/kg/min (Figure 2). After the discontinuation of epoprostenol, the estimated right ventricular pressure as perultrasound cardiography was approximately 60% of the left ventricular pressure (Figure 2).