Table 2.
Cardiopulmonary Characteristics of Subjects with SSc-ID and PH
| Cardiopulmonary Characteristics | SSc-ILD and PH (N=29) |
|---|---|
| WHO PH Group, no. (%) | |
| WHO Group 1 | 7 (24.1) |
| WHO Group 2 | 6 (20.7) |
| WHO Group 3 | 16 (55.2) |
| ILD Involvement on HRCT, no. (%) | |
| > 20% | 19 (65.5) |
| < 20% | 10 (34.5) |
| PFTs (at PH Diagnosis), mean ± SD | |
| FVC% Predicted | 70.3 ± 18.1 |
| TLC % predicted | 84.7 ± 16.5 |
| DLco % Predicted | 43.1 ± 15.8 |
| FVC% Predicted / DLco% Predicted | 2 ± 1.5 |
| TTE (at PH Diagnosis), mean ± SD | |
| RVSP (mmHg) N=27* | 44.9 ± 21.6 |
| RAP (mmHg) N=27* | 7.9 ± 3.2 |
| RA Dilation, no. (%) | 13 (44.8) |
| RV Dilation, no. (%) | 11 (37.9) |
| Abnormal RV Function, no. (%) N=28 | 6 (21.4) |
| RHC, mean ± SD | |
| mPAP mmHg | 33.4 ± 7.2 |
| mPCWP mmHg | 13.2 ± 3.2 |
| mRAP mmHg | 9.9 ± 3.3 |
| CO (Fick) | 5.3 ± 1.5 |
| CO (TD) | 5.3 ± 1.5 |
| PVR (WU) | 4.3 ± 3.3 |
| Ranges of mPAP on RHC, no. (%) | |
| mPAP 25 – 35 mmHg | 20 (69) |
| mPAP 35 – 45 mmHg | 8 (27.6) |
| mPAP > 45 mmHg | 1 (3.4) |
| Ranges of PVR on RHC, no (%) | |
| PVR 0 – 6 WU | 21 (72.4) |
| PVR 6 – 12 WU | 7 (24.1) |
| PVR > 12 WU | 1 (3.4) |
No tricuspid regurgitation jet observed in 2 subjects
SSc- systemic sclerosis; ILD- interstitial lung disease; SSc-ILD- systemic sclerosis associated interstitial lung disease; PH- pulmonary hypertension; WHO - World Health Organization; HRCT- high resolution computed tomography; PFTs- pulmonary function tests; FVC- forced vital capacity; TLC- total lung capacity; DLCO- diffusing capacity for carbon monoxide; TTE- transthoracic echocardiography; RVSP- right ventricular systolic pressure; RAP –right atrial pressure; RA- right atrial; RV- right ventricular; RHC – right heart catheterization; mPAP – mean pulmonary arterial pressure; PCWP- pulmonary capillary wedge pressure; CO- cardiac output; TD- thermodilution; PVR – pulmonary vascular resistance on RHC; WU- woods units.