Overview of characteristics of individuals diagnosed with pulmonary hypertension in the NPHU between 2010 and 2020
| Group 1 | Group 2 | Group 3 | Group 4 | Group 5 | Mixed | |
| Subjects, n | 163 | 69 | 39 | 67 | 23 | 54 |
| Sex: female n (%) | 125 (77) | 45 (65) | 20 (51) | 29 (43) | 9 (39) | 26 (48) |
| Age (years): mean±SD | 56±15 | 69±10 | 64±13 | 61±16 | 57±13 | 64±11 |
| WHO functional class: % I/II/III/IV | 1/29/56/14 | 1/26/49/22 | 0/15/56/28 | 1/28/64/7 | 0/22/74/4 | 0/22/61/17 |
| BNP (ng/L): median (IQR) | 172 (57–570) | 366 (234–826) | 117 (41–468) | 128 (62–451) | 90 (45–183) | 225 (78–860) |
| Right heart catheterisation | ||||||
| mRAP (mm Hg) | 9±6 | 11±6 | 6±3 | 9±5 | 8±6 | 11±6 |
| mPAP (mm Hg) | 46±15 | 42±12 | 39±11 | 44±9 | 39±12 | 40±12 |
| PAWP (mm Hg) | 11±5 | 20±7 | 10±5 | 11±4 | 12±6 | 15±7 |
| CO (L/min) | 4±1 | 5±1 | 4±2 | 4±1 | 5±1 | 4±1 |
| PVR (WU) | 10±7 | 5±3 | 7±4 | 9±4 | 6±5 | 7±5 |
| DLCO, % predicted | 49±23 | 46±15 | 35±14 | 67±13 | 42±18 | 41±16 |
| PH therapy within 12 months of diagnosis: n (%) | 148 (91) | 33 (48) | 29 (74) | 54 (81) | 17 (74) | 35 (65) |
| Monotherapy | 37 (23) | 29 (42) | 23 (59) | 38 (57) | 8 (35) | 26 (48) |
| Double combination therapy | 88 (54) | 4 (6) | 5 (13) | 15 (22) | 7 (30) | 7 (13) |
| Triple combination therapy | 23 (14) | 0 | 1 (3) | 1 (1) | 2 (9) | 1 (2) |
| Balloon atrial septostomy | 3 (2) | 0 | 0 | 0 | 0 | 0 |
| Lung transplantation | 5 (3) | 0 | 2 (5) | 0 | 1 (4) | 0 |
| Survival* | ||||||
| 1-year, 3-year and 5- year survival, % | 89, 75, 65 | 94, 88, 75 | 66, 33, 14 | 93, 79, 64 | 100, 75, 75 | 89, 71, 64 |
| Median follow-up (IQR): years | 3 (1–5) | 2 (1–4) | 1.5 (1–3) | 3 (2–5) | 2 (1–3) | 3 (1–4) |
FC, BNP, RHC parameters and lung function were collected at diagnosis. WHO FC was available for all 415 patients. Of note data were incomplete for the following parameters: BNP not available for 12%, mRAP in 39%, mPAP in 9%, PAWP in 19%, CO in 46%, PVR in 54% and DLCO in 52%.
Survival data were available for 19 (86%) individuals with CHD-PAH.
BNP, B-type natriuretic peptide; CO, cardiac output; DLCO, diffusion capacity for carbon monoxide; mPAP, mean pulmonary artery pressure; mRAP, mean right atrial pressure; PAWP, pulmonary artery wedge pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance.;