|
|
Sildenafil Dose |
|
Placebo (n=60) |
Low
(n=42) |
Medium
(n=55) |
High
(n=77) |
Combined
(n=174) |
| Female sex, n (%) |
38 (63) |
25 (60) |
31 (56) |
51 (66) |
107 (62) |
| Age in years, n (%) |
|
|
|
|
|
| 1–4 |
7 (11) |
0 (0) |
9 (16) |
19 (25) |
28 (16) |
| 5–12 |
37 (62) |
25 (60) |
28 (51) |
36 (47) |
89 (51) |
| 13–17 |
16 (27) |
17 (40) |
18 (33) |
22 (29) |
57 (33) |
| WHO functional class, n (%) |
|
|
|
|
|
| I |
25 (42) |
9 (21) |
20 (36) |
21 (27) |
50 (29) |
| II |
29 (48) |
23 (55) |
25 (45) |
43 (56) |
91 (52) |
| III |
6 (10) |
9 (21) |
8 (15) |
12 (16) |
29 (17) |
| IV |
0 |
0 |
1 (2) |
0 |
1 (1) |
| Missing |
0 |
1 (2) |
1 (2) |
1 (1) |
3 (2) |
| Etiology, n (%) |
|
|
|
|
/td> |
| IPAH/FPAH |
21 (35) |
12 (29) |
19 (35) |
26 (34) |
57 (33) |
| PAH-CHD |
39 (65) |
30 (71) |
36 (65) |
51 (66) |
117 (67) |
| PAH-related variables, mean (SD) |
|
|
|
|
|
| Peak VO2, mL/kg/min‡ |
20 (4) |
18 (4) |
18 (5) |
17 (4) |
18 (4) |
| Mean pulmonary artery pressure, mmHg‡ |
59 (22) |
66 (23) |
62 (18) |
62 (24) |
63 (22) |
| Cardiac index, L/min/m2§ |
3.9 (2.1) |
3.1 (1.1) |
3.3 (1.5) |
3.4 (1.6) |
3.3 (1.5) |
| Pulmonary vascular resistance index, Wood units•m2║ |
15 (10) |
22 (13) |
19 (14) |
20 (16) |
20 (15) |
Note: CHD=congenital heart disease; FPAH=familial PAH; IPAH=idiopathic PAH; PAH=pulmonary arterial hypertension; VO2=oxygen consumption; WHO=World Health Organization.
*The groups shown represent all treated patients.
‡Subset of patients developmentally able to perform exercise testing (n=30, 28, 28, 29, and 85 for placebo, sildenafil low-, medium-, and high-dose groups, and sildenafil
combined dose group, respectively).
‡n=59, 42, 55, 75, and 172 for placebo, sildenafil low-, medium-, and high-dose groups, and sildenafil combined dose group, respectively.
§ n=59, 41, 52, 74, and 167 for placebo, sildenafil low-, medium-, and high-dose groups, and sildenafil combined dose group, respectively.
║n=57, 40, 52, 73, and 165 for placebo, sildenafil low-, medium-, and high-dose groups, and sildenafil combined dose group, respectively. |
