You are viewing the site in preview mode
Skip to main content
| |
All
|
IPAH
|
APAH**
|
FPAH
|
Other***
|
|---|
|
Total, n (%)
|
4241
|
2319 (54.6)
|
1479 (34.8)
|
252 (5.9)
|
191 (4.6)
|
|
Sex, n (%)
|
|
F
|
3187 (75.1)
|
1721 (74.2)
|
1156 (78.2)
|
173 (68.6)
|
137 (71.7)
|
|
M
|
1054 (24.9)
|
598 (25.8)
|
323 (21.8)
|
79 (31.3)
|
54 (28.3)
|
|
F:M ratio
|
3:1
|
2.9:1
|
3.6:1
|
2.3:1
|
2.5:1
|
|
Age-of-onset, n (%)
|
|
Adult (≥ 18 years)
|
3780 (89.2)
|
2126 (91.7)
|
1256 (85.2)
|
213 (84.5)
|
185 (96.9)
|
|
Child (< 18 years)
|
457 (10.8)
|
193 (8.3)
|
219 (14.8)
|
39 (15.4)
|
6 (3.1)
|
|
Mean ± SD
|
45.9 ± 20.0
|
47.0 ± 19.5
|
45.2 ± 21.3
|
36.8 ± 16.8
|
47.7 ± 15.0
|
|
Ancestry, n (%)
| | | | | |
|
EUR
|
3108 (74.5)
|
1798 (77.5)
|
988 (67.0)
|
166 (86.9)
|
156 (81.7)
|
|
HISP
|
359 (8.6)
|
145 (6.3)
|
186 (12.6)
|
13 (6.8)
|
15 (7.9)
|
|
AFR
|
365 (8.7)
|
166 (7.2)
|
189 (12.8)
|
2 (1.0)
|
8 (4.2)
|
|
EAS
|
104 (2.5)
|
42 (1.8)
|
57 (3.9)
|
1 (0.5)
|
4 (2.1)
|
|
SAS
|
116 (2.8)
|
82 (3.5)
|
28 (1.9)
|
4 (2.1)
|
2 (1.0)
|
|
Other/unknown
|
123 (2.9)
|
85 (3.7)
|
27 (1.8)
|
5 (2.6)
|
6 (3.1)
|
|
Hemodynamic parameters, mean ± SD (n)
|
|
MPAP, mmHg
|
51 ± 14 (3594)
|
53 ± 17 (2045)
|
48 ± 14 (1235)
|
58 ± 14 (158)
|
51 ± 12 (156)
|
|
BMPR2+
|
59 ± 12 (320)
|
60 ± 12 (197)
|
56 ± 16 (11)
|
58 ± 12 (109)
|
53 ± 5 (3)
|
|
BMPR2−
|
50 ± 14 (3394)
|
52 ± 14 (1928)
|
48 ± 14 (1257)
|
57 ± 17 (65)
|
51 ± 13 (144)
|
|
MPCWP, mmHg
|
10 ± 4 (3407)
|
10 ± 24 (1912)
|
10 ± 4 (1197)
|
10 ± 4 (151)
|
11 ± 4 (147)
|
|
BMPR2+
|
10 ± 4 ()
|
10 ± 4 ()
|
10 ± 3 ()
|
10 ± 4 ()
|
8 ± 4 ()
|
|
BMPR2−
|
10 ± 4 ()
|
10 ± 4 ()
|
10 ± 4 ()
|
10 ± 4 ()
|
11 ± 4 ()
|
- *US/UK PAH cohort: 2572 PAH Biobank; 1134 UK NIHR BioResource; 534 CUIMC
- **APAH: PAH associated with connective tissue diseases, congenital heart disease, HHT, HIV
- ***Other: diet- and toxin-induced PAH, non-familial pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis and one case of persistent pulmonary hypertension of the newborn
- Abbreviations: AFR, African; EAS, East Asian; EUR, European; HISP, Hispanic; SAS, Southeast Asian; MPAP, mean pulmonary artery pressure; MPCWP, mean pulmonary capillary wedge pressure
