| |
All
|
IPAH
|
APAH
|
FPAH
|
Other*
|
|---|
|
Total (%)
|
2572
|
1110 (43.2)
|
1239 (48.2)
|
101 (3.9)
|
122 (4.7)
|
|
Age of onset, n (%)
|
|
Child (dx age < 19)
|
226 (8.8)
|
94 (8.5)
|
112 (9.0)
|
15 (14.9)
|
5 (4.1)
|
|
Adult (dx age > =19)
|
2345 (91.2)
|
1015 (91.4)
|
1127 (91.0)
|
86 (85.1)
|
117 (95.9)
|
|
Mean age
|
48 ± 19
|
48 ± 18
|
49 ± 19
|
37 ± 15
|
47 ± 15
|
|
Gender, n (%)
|
|
Female
|
2023 (78.7)
|
868 (78.2)
|
996 (80.4)
|
69 (68.3)
|
90 (73.8)
|
|
Male
|
549 (21.3)
|
242 (21.8)
|
243 (19.6)
|
32 (31.7)
|
32 (26.2)
|
|
Female to male ratio
|
3.7:1
|
3.6:1
|
4.1:1
|
2.2:1
|
2.8:1
|
|
Ancestry, n (%)
|
|
European
|
1852 (72)
|
809 (73.0)
|
855 (69.0)
|
89 (88.1)
|
99 (81.2)
|
|
Hispanic
|
315 (12.3)
|
137 (12.3)
|
156 (12.6)
|
10 (9.9)
|
12 (9.8)
|
|
African
|
292 (11.4)
|
117 (10.5)
|
168 (13.6)
|
1 (1)
|
6 (4.9)
|
|
East Asian
|
70 (2.7)
|
25 (2.2)
|
41 (3.3)
|
0
|
4 (3.3)
|
|
South Asian
|
28 (1.1)
|
12 (1.1)
|
15 (1.2)
|
1 (1)
|
0
|
|
Others
|
15 (0.58)
|
10 (0.9)
|
4 (0.3)
|
0
|
1 (0.8)
|
|
Hemodynamic parameters
|
|
MPAP (mmHg)
|
50 ± 14
|
52 ± 14
|
48 ± 14
|
58 ± 14
|
52 ± 13
|
|
MPCW (mmHg)
|
10 ± 4
|
10 ± 4
|
10 ± 4
|
10 ± 4
|
11 ± 4
|
|
CO, Fick (L/min)
|
4.5 ± 1.8
|
4.5 ± 1.7
|
4.6 ± 1.9
|
3.6 ± 1.0
|
4.2 ± 1.3
|
|
PVR (Woods units)
|
10.7 ± 7.0
|
11.2 ± 7.0
|
10.0 ± 7.1
|
14.9 ± 6.3
|
11.0 ± 6.6
|
|
MAP (mmHg)
|
90 ± 19
|
91 ± 20
|
90 ± 19
|
88 ± 16
|
94 ± 19
|
|
MAP:MPAP
|
1.9 ± 0.7
|
1.9 ± 0.7
|
2.0 ± 0.7
|
1.6 ± 0.5
|
1.9 ± 0.5
|
- Abbreviations: MPAP mean pulmonary arterial pressure, MPCW mean pulmonary capillary wedge pressure, CO cardiac output by Frick’s method, PVR pulmonary vascular resistance, MAP mean arterial pressure
- *Other included 110 diet- and toxin-induced PAH, 11 non-familial pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis, and 1 persistent pulmonary hypertension of the newborn
