Table 5

Patient Characteristics Stratified for Therapy

IPAH/FPAH (n = 212)CCB ± PAH-Targeted
(n = 31)
PAH-Targeted
(n = 163)
No Therapy
(n = 18)
p Value
Age at diagnosis, yrs7.3 (4.6–12.9)8.0 (3.9–13.2)6.0 (4.2–13.1)0.833
Female26 (83.9)94 (57.7)13 (72.2)0.008
Incident patients14 (45.2)82 (50.3)10 (55.6)0.560
WHO functional class
 I3 (9.7)19 (11.7)6 (33.3)0.280
 II18 (58.1)67 (41.1)4 (22.2)
 III7 (22.6)59 (36.2)7 (38.9)
 IV3 (9.7)18 (11.0)1 (5.6)
WHO functional class
 I and II21 (67.7)86 (52.8)10 (55.6)0.128
 III and IV10 (32.3)77 (47.2)8 (44.4)
6-min walking distance, min414 ± 128397 ± 127358 ± 1040.557
Baseline mPAP, mm Hg50 ± 1661 ± 1862 ± 180.002
Baseline mRAP, mm Hg5 ± 37 ± 48 ± 4<0.001
Baseline PVRi, WU·m213.0 (7.4–18.6)15.9 (10.9–22.9)21.3 (8.0–26.5)0.014
Baseline cardiac index, l/min/m23.3 (2.9–4.3)2.9 (2.4–4.0)3.0 (2.0–4.0)0.133
Baseline PVRi/SVRi0.6 (0.4–0.8)0.9 (0.7–1.1)0.8 (0.7–1.1)<0.001
Baseline mPAP/mSAP0.7 (0.5–0.8)0.9 (0.7–1.1)0.9 (0.7–1.1)<0.001
AVT mPAP, mm Hg29 ± 1452 ± 1953 ± 20<0.001
AVT mRAP, mm Hg5 ± 47 ± 48 ± 30.003
AVT PVRi, WU·m24.1 (3.2–5.9)12.4 (7.0–19.7)13.6 (8.2–22.9)<0.001
AVT cardiac index, l/min/m24.1 (3.4–5.1)3.1 (2.4–4.1)3.1 (2.4–4.1)<0.001
AVT PVRi/SVRi0.3 (0.2–0.4)0.7 (0.4–1.0)0.6 (0.4–0.9)<0.001
AVT mPAP/mSAP0.3 (0.3–0.5)0.7 (0.5–1.0)0.8 (0.5–1.1)<0.001

Values are median (interquartile range), n (%), or mean ± SD.

CCB = calcium-channel blocker; other abbreviations as in Tables 1, 2, and 4.

  • The p value of patients on CCB ± PAH-targeted therapy (the CCB monotherapy group combined with the CCB and PAH-targeted therapy group) versus the other patients.