Outcome in children with IPAH/HPAH
| Potts shunt n=8 | No Potts shunt n=9 | |
| Death (all cause)/LTx n | 2/0 | 0/2 |
| Survivors | ||
| n=6 | n=7 | |
| Median follow-up years | ||
| Since initiation of upfront triple combination therapy | 7.3 (2.4–8.3) | 2.5 (1.5–4.0) |
| Since Potts procedure | 2.6 (0.5–7.0) | NA |
| Prostanoids stopped n | 5 (83) | 2 (29) |
| WHO-FC I/II | 6 (100) | 6 (86) |
| Clinical symptoms of right heart failure yes | 0 | 0 |
| NT-proBNP ng·L−1 | 70 (61–179) | 66 (46–236) |
| NT-proBNP <300 ng·L−1 | 5 (83) | 6 (86) |
| Poor systolic RV function | 0 | 0 |
| Pericardial effusion yes | 0 | 0 |
Data are presented as median (interquartile range) or n (%). PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; LTx: lung transplantation; WHO-FC: World Health Organization Functional Class; NT-proBNP: N-terminal pro-brain-natriuretic peptide; RV: right ventricle; NA: not applicable.