1-year, 3-year and 5-year survival of individuals with PAH, with a specific focus on CTD-PAH, IPAH, CHD-PAH and PoPH
| PAH | CTD-PAH | IPAH | CHD-PAH | PoPH | |
| Subjects, n | 160* | 80 | 33 | 19* | 11 |
| Annual incidence | 3.11 | 1.53 | 0.63 | 0.63* | 0.21 |
| Survival, % | |||||
| 1 year | 89 | 83 | 100 | 100 | 91 |
| 3 year | 75 | 62 | 100 | 100 | 81 |
| 5 year | 65 | 47 | 95 | 91 | 81 |
| Median (IQR) follow-up, years | 3 (1–5) | 2.5 (1–4) | 3 (2–7) | 4 (3–7) | 5 (3–6.5) |
The median follow-up in years and IQR is also provided for each subgroup.
*The calculated incidence for CHD-PAH does not reflect the true incidence of this particular subgroup as these patients are an exception in that they are not consistently referred to the NPHU. Survival data were not available for three subjects with CHD-PAH. Annual incidence is reported per million population (pmp).
CHD-PAH, congenital heart disease PAH; CTD-PAH, connective tissue disease PAH; IPAH, idiopathic pulmonary arterial hypertension; PAH, pulmonary arterial hypertension; PoPH, portopulmonary pulmonary hypertension.