Diagnosis | 3-year follow-up | 4-year follow-up | 5-year follow-up | |
Subjects n | 386 | 251 | 193 | 139 |
Age years | 60 (43–70) | 60 (43–72) | 60 (42–72) | 58 (40–71) |
Female sex % | 68 | 69 | 69 | 71 |
PAH subsets % | ||||
IPAH/FPAH | 49 | 52 | 51 | 49 |
APAH-CTD | 30 | 26 | 23 | 22 |
APAH-CHD | 13 | 13 | 17 | 19 |
APAH-Others | 9 | 9 | 9 | 10 |
Comorbidities % | ||||
Hypertension | 31 | 31 | 25 | 21 |
Diabetes mellitus | 13 | 13 | 10 | 8 |
Atrial fibrillation | 9 | 8 | 8 | 8 |
Previous stroke | 4 | 4 | 2 | 4 |
Ischaemic heart disease | 10 | 11 | 9 | 7 |
Thyroid disease | 12 | 14 | 12 | 14 |
Obesity (BMI >30 kg·m−2) | 19 | 21 | 15 | 14 |
Kidney dysfunction (eGFR <60 mL·kg−1·m−2) | 27 | 34 | 26 | 21 |
PAH-targeted treatment % | ||||
ERA | 50 | 20 | 23 | 22 |
PDE5 inhibitor | 20 | 13 | 15 | 12 |
Prostacyclin | 3 | 1 | 1 | 1 |
sGC stimulator | 0 | 0 | 0 | 0 |
Study drug | 3 | 1 | 0 | 0 |
Dual therapy | 12 | 48 | 43 | 42 |
Triple therapy | 1 | 10 | 10 | 14 |
Quadruple therapy | 0 | 0 | 1 | 0 |
No treatment registered | 12 | 7 | 8 | 7 |
Supportive therapy % | ||||
Anticoagulants | 58 | 61 | 64 | 62 |
Diuretics | 56 | 65 | 59 | 57 |
Supplemental oxygen | 17 | 26 | 22 | 18 |
Risk group % | ||||
Low risk | 31 | 39 | 48 | 47 |
Intermediate risk | 62 | 54 | 45 | 49 |
High risk | 7 | 8 | 8 | 4 |
Data are presented as median (interquartile range), unless otherwise stated. PAH: pulmonary arterial hypertension; APAH: associated pulmonary arterial hypertension; IPAH: idiopathic pulmonary arterial hypertension; FPAH: familial pulmonary arterial hypertension; CTD: connective tissue disease; CHD: congenital heart disease; BMI: body mass index; eGFR: estimated glomerular filtration rate; ERA: endothelin receptor antagonist; PDE5: phosphodiesterase 5; sGC: soluble guanylate cyclase.