Table 2.
Baseline clinical, radiological, lung function, and right heart catheterization data
| Measure (N=number of pts; Normal/Borderline/Resting PH) | TOTAL(N=206) | NORMAL(N=35) | BORDERLINE (N=28) | Resting PH (N=143) | ||||
|---|---|---|---|---|---|---|---|---|
| Mean(SD) | Median | Mean(SD) | Median | Mean(SD) | Median | Mean(SD) | Median | |
| NYHA classification, N(%)(N=27/26/125) | ||||||||
| 1=Dyspnea with extreme activity | 40(22.4) | 9(33.3) | 8(30.8) | 23(18.4) | ||||
| 2=Dyspnea with moderate activity | 79(44.4) | 15(55.6) | 12(46.2) | 52(41.6) | ||||
| 3=Dyspnea with minimal activity | 59(33.1) | 3(11.1) | 6(23.1) | 50(40.0) | ||||
| Modified Rodnan skin score | 9.1(8.5) | 6.0 | 8.8(8.7) | 5.0 | 7.7(6.0) | 5.5 | 9.5(8.8) | 6.0 |
| Autoantibodies, N(%) (N=34/23/128) | ||||||||
| Anticentromere ab | 43(15.7) | 4(11.8) | 4(17.4) | 35(27.3) | ||||
| Anti-Scl 70 ab | 29(15.7) | 8(23.5) | 4(17.4) | 17(13.3) | ||||
| Anti-U1RNP ab | 14(7.6) | 5(14.7) | 2(8.7) | 7(5.5) | ||||
| Anti-RNA Polymerase III ab | 5(2.7) | 1(2.9) | 0(0) | 4(3.1) | ||||
| ANA Isolated nucleolar pattern | 49(26.5) | 9(26.5) | 5(21.7) | 35(27.3) | ||||
| ANA Mixed or other staining pattern | 32(17.3) | 4(11.8) | 4(17.4) | 24(18.8) | ||||
| ANA Negative | 13(7.0) | 3(8.8) | 4(17.4) | 6(4.7) | ||||
| Pulmonary Function Test (N=30/24/128) | ||||||||
| FVC % predicted | 74.7(20.1) | 75.8 | 81.9 (18.9) | 82.8 | 69.3 (16.1) † | 66.0 | 74.1(20.7) | 76.3 |
| FVC<65% predicted, N (%) | 59(32.4) | 7(23.3) | 10(41.7) | 42(32.8) | ||||
| FVC< 70% predicted, N (%) | 74(40.7) | 9(30.0) | 16 (66.7) † | 49(38.3) | ||||
| DLco % predicted | 40.6(16.1) | 37.7 | 45.7 (10.9) | 47.5 | 40.5 (18.1) | 38.2 | 39.4(16.6) | 36.6 |
| FVC/DLco | 2.1(0.9) | 1.9 | 1.8(0.3) | 1.9 | 2.1(1.4) | 1.9 | 2.1(0.9) | 2.0 |
| HRCT fibrosis, N (%)(N=24/20/88) | ||||||||
| None | 48(36.4) | 9(37.5) | 4(20.0) | 35(39.8) | ||||
| Mild | 36(27.2) | 9(37.5) | 8(40.0) | 19(21.6) | ||||
| Moderate | 33(25.0) | 6(25.0) | 6(30.0) | 21(23.9) | ||||
| Severe | 15(11.4) | 0(0) | 2(10.0) | 13(14.8) | ||||
| 6 minute walk test, meters (N=25/23/109) | 353.7(129.9) | 384.0 | 454.3 (82.6) | 457.2 | 393.3(121.5) | 405.8 | 321.6(127.0) | 341.9 |
| Echo RVSP, mmHg (N=31/22/129)¶ | 53.0(21.0) | 50.0 | 36 .2(8.7) | 35.0 | 46.3 (13.6) ‡ | 44.0 | 58.1(21.8) | 54.0 |
| Right Heart Catheterization (RHC) | ||||||||
| mPAP, mmHg | 31(12) | 29 | 17 (3) | 17 | 24 (5) ‡ | 23 | 36(11) | 31 |
| PCWP, mmHg | 11(5) | 10 | 8 (3) | 8 | 10 (3) ‡ | 10 | 12(5) | 11 |
| CO, L/min | 5.2(1.5) | 5.1 | 5.7 (1.2) | 5.8 | 5.4 (1.3) | 5.3 | 5.1(1.6) | 5.0 |
| PVR, dyn*sec*cm-5 | 345(251) | 258 | 137(74) | 126 | 210(110) ‡ | 177 | 422(260) | 348 |
| TPG, mmHg | 20(12) | 16 | 9 (3) | 9 | 13 (6) ‡ | 13 | 24(12) | 21 |
| Exercise RHC(N=18/16/25) | ||||||||
| mPAP, mmHg | 37(10) | 37 | 31(7) | 31 | 39(7) † | 40 | 41(11) | 39 |
| PCWP, mmHg | 14(7) | 14 | 13(6) | 11 | 17(9) | 15 | 13(6) | 14 |
| CO, L/min | 7.5(2.5) | 7.0 | 8.7(2.6) | 7.9 | 7.7(1.9) | 7.2 | 6.7(2.5) | 6.4 |
| PVR, dyn*s* cm−5 | 295(215) | 215 | 175(74) | 192 | 255(215) | 212 | 391(235) | 358 |
†
p< 0.05;
‡
p< 0.002 between Normal and boPAP,
¶
Echo RVSP is the same as echo estimate sPAP in case of the absence of pulmonary valvular stenosis, FVC forced vital capacity; Echo RVSP Right ventricular systolic pressure on echocardiogram; mPAP mean pulmonary arterial pressure; PCWP pulmonary capillary wedge pressure; CO cardiac output; PVR pulmonary vascular resistance; TPG transpulmonary gradient.