Adult PAH | | | |
Subjects | 136 | 32# | |
Age at diagnosis years (n=168) | 54.0±18 | 42.5±12.3 | <0.0001§ |
Minimum–maximum | 18–86 | 20–69 | |
Female/male | 93 (68)/43 (32) | 24 (75)/8 (25) | 0.527 |
NYHA functional class (n=156) | | | |
I/II | 33 (26) | 6 (21) | 0.641 |
III/IV | 94 (74) | 23 (79) | |
Mean pulmonary arterial pressure mmHg (n=156) | 50.2±13.5 | 57.4±13.1 | 0.004§ |
Pulmonary vascular resistance Wood Units (n=146) | 11.6±6.2 | 14.4±7.3 | 0.036 |
Right atrial pressure mmHg (n=151) | 8.3±5.2 | 9.6±6.2 | 0.385 |
Cardiac output L·min−1 (n=155) | 4.2±1.5 | 3.8±1.4 | 0.056 |
Cardiac index L·min−1·m−2 (n=153) | 2.3±0.8 | 2.2±0.9 | 0.086 |
Vasodilator responder (n=112) | 12 (13.5) | 1 (4.3) | 0.297 |
Paediatric PAH | | | |
Subjects | 26 | 9¶ | |
Age at diagnosis years (n=35) | 5.4±5.3 | 10±3.7 | 0.020 |
Minimum–maximum | (0.035–16) | (4.5–15) | |
Median (IQR) | 4.1 (6.9) | 10.2 (5.2) | |
Female/male | 15 (58)/11 (42) | 6 (67)/3 (33) | 0.712 |
NYHA functional class (n=25) | | | |
I/II | 7 (41) | 5 (62) | 0.411 |
III/IV | 10 (59) | 3 (38) | |
Mean pulmonary arterial pressure mmHg (n=29) | 51±17.7 | 55.1±15.8 | 0.494 |
Median (IQR) | 51 (23) | 54.5 (15.7) | |
Pulmonary vascular resistance Wood Units (n=27) | 22.3±18.6 | 13.6±6.8 | 0.300 |
Median (IQR) | 17.3 (17.3) | 14 (7.6) | |
Right atrial pressure mmHg (n=25) | 6.2±3.4 | 5.3±1.8 | 0.807 |
Median (IQR) | 5.5 (4) | 5 (1) | |
Cardiac output L·min−1 (n=25) | 2.6±1.2 | 4.5±3 | 0.066 |
Median (IQR) | 2.5 (1.4) | 3.2 (1.3) | |
Cardiac index L·min−1·m−2 (n=24) | 3.8±1.3 | 3.6±1.2 | 0.854 |
Median (IQR) | 3.3 (1.4) | 3.1 (1.1) | |
Acute vasodilator responder (n=26) | 6 (33) | 1 (12) | 0.375 |
Adult PVOD | | | |
Subjects | 49 | 7+ | |
Age at diagnosis years (n=56) | 61±13.2 | 34.4±13.1 | <0.0001§ |
Minimum–maximum | 22–88 | 21–52 | |
Median (IQR) | 64 (13) | 35 (21.5) | |
Female/male | 19 (39)/30 (61) | 3 (43)/4 (57) | 1 |
NYHA functional class (n=47) | | | |
I/II | 11 (25) | 2 (67) | 0.181 |
III/IV | 33 (75) | 1 (33) | |
Mean pulmonary arterial pressure mmHg (n=48) | 47.3±11.8 | 47.7±20.3 | 0.614 |
Median (IQR) | 47.5 (17.2) | 38.5 (14.2) | |
Pulmonary vascular resistance Wood Units (n=46) | 10.2±9.4 | 9.9±6.2 | 0.770 |
Median (IQR) | 8.7 (4.3) | 7.2 (4.4) | |
Right atrial pressure mmHg (n=45) | 8±5.1 | 5.2±2.1 | 0.336 |
Median (IQR) | 7 (6) | 5.5 (3.2) | |
Cardiac output L·min−1 (n=46) | 4.4±1.1 | 3.8±0.4 | 0.250 |
Median (IQR) | 4.5 (1.3) | 3.8 (0.5) | |
Cardiac index L·min−1·m−2 (n=47) | 2.4±0.6 | 2.3±0.2 | 0.542 |
Median (IQR) | 2.4 (0.8) | 2.2 (0.2) | |
Solvent exposure or previous chemotherapy (n=40) | 17 (42.5) | 0 (0) | 0.014 |