Table 1.
CTD-associated PAH
|
Idiopathic PAH (n = 1,654) | P, all CTD-associated PAH vs. idiopathic PAH | |||
---|---|---|---|---|---|
All (n = 716) | SSc-associated PAH (n = 407) | Non–SSc-associated PAH (n = 309) | |||
Age, years | 53 ± 14 | 59 ± 11 | 45 ± 14 | 46 ± 15 | <0.001 |
Female sex, % | 89 | 88 | 90 | 73 | <0.001 |
Race, % | 0.39 | ||||
White | 71 | 88 | 49 | 73 | |
Black | 5 | 4 | 6 | 4 | |
Other | 25 | 8 | 45 | 23 | |
Body mass index, kg/m2 | 26 ± 6 | 27 ± 6 | 26 ± 6 | 28 ± 7 | <0.001 |
WHO functional class, % | 0.18 | ||||
I–II | 47 | 37 | 59 | 44 | |
III–IV | 53 | 63 | 41 | 56 | |
Baseline hemodynamics | |||||
Right atrial pressure, mm Hg | 8.0 ± 5.0 | 8.3 ± 4.9 | 7.7 ± 5.0 | 9.6 ± 5.8 | <0.001 |
Mean pulmonary arterial pressure, mm Hg | 46 ± 12 | 45 ± 13 | 48 ± 12 | 56 ± 16 | <0.001 |
Cardiac index, liters/minute/m2 | 2.5 ± 0.8 | 2.5 ± 0.8 | 2.5 ± 0.8 | 2.3 ± 0.8 | <0.001 |
Pulmonary capillary wedge pressure, mm Hg | 9 ± 4 | 10 ± 3 | 9 ± 3 | 9 ± 4 | 0.70 |
Pulmonary vascular resistance, Wood units | 10.2 ± 6.2 | 9.6 ± 6.4 | 10.9 ± 5.9 | 13.3 ± 7.8 | <0.001 |
Baseline 6-minute walk distance, meters | 336 ± 93 | 322 ± 92 | 353 ± 91 | 347 ± 89 | <0.001 |
Drug class, % | 0.021 | ||||
Endothelin receptor antagonists | 71 | 72 | 69 | 67 | |
Phosphodiesterase 5 inhibitors | 12 | 13 | 11 | 13 | |
Prostacyclin analogs | 17 | 16 | 20 | 22 |
Except where indicated otherwise, values are the mean ± SD.
CTD-associated PAH = connective tissue disease–associated pulmonary arterial hypertension; SSc = systemic sclerosis; WHO = World Health Organization.