Characteristics and metaanalysis of studies of patients with SSc and pulmonary hypertension. Values are mean ± SD or number (%) of observations, unless otherwise specified.
| Current Study | Mukerjee10 | Hachulla11 | Vonk30 | Phung31 | Metaanalysis, % (95% CI) | |
|---|---|---|---|---|---|---|
| Indications for RHC | sPAP > 40 mm Hg on echocardiography, OR DLCO < 50% predicted without pulmonary fibrosis, OR unexplained dyspnea | sPAP > 35 mm Hg, OR DLCO < 50% predicted without pulmonary fibrosis, OR breathlessness without pulmonary fibrosis | VTR > 3 m/s OR 2.5–3 m/s + unexplained dyspnea. Patients with FVC, DLCO, or FEV1 < 60% predicted were excluded | sPAP > 35 mm Hg | Estimated RVSP > 40 mm Hg, OR symptoms with RVSP of 35–40 mm Hg, OR echocardiographic increase in PAP (if RVSP could not be measured) | |
| Total population | 1165 | 722 | 599 | 1148 | 184 | 3818 |
| Patients with precapillary PH | 64 (5) | 89 (12) | 47 (8) (29 with known PAH and 18 newly diagnosed) | 113 (10) | 24 (13) | 9 (7–12) |
| Age of patients with precapillary PH, yrs | 68 ± 14 | 66 ± 7 | 65 ± 12 | 67 (median) | 67 (median) | 66.5 ± 12 |
| Disease duration in patients with precapillary PH, yrs | 15 ± 9 | 14 ± 5 | 11.9 + 12.6 | NA | 11 (median) | 13 ± 9 |
| Men | 14/64 | 28/148 | 2/18 | NA | 3/24 | 18 (12–26) |
| Pulmonary fibrosis | 29/64 | 40/148 | NA | NA | 6/24 | 32 (26–38) |
| Limited cutaneous SSc | 42/64 | 111/148 | 10/18 | NA | 20/24 | 72 (66–77) |
| Pulmonary arterial hypertension | 42/64 | NA | NA | 35/57 | NA | 63 (55–72) |
| FVC < 70% predicted | 22/64 | NA | NA | 22/57 | NA | 36 (28–45) |
| Suspected PH leading to RHC | 206/1165 (18) | 147/722 (20) | 33/599 (6) | NA | 44/184 (24) | 13 (7–23) |
| Causes of noncontributory RHC | 123 normal RHC 17 left-heart disease 2 pulmonary venoocclusive disease | 36 normal RHC 19 left-heart disease 3 thromboembolic PH | 12 normal RHC 3 left-heart disease | NA | 20 normal RHC | Noncontributive: 55% (50–59) Normal: 44% (40–49) Left-heart disease: 9% (7–12) |
PH: pulmonary hypertension: RHC: right-heart catheterization; RVSP: right ventricular systolic pressure; sPAP: systolic pulmonary artery pressure; DLCO: carbon monoxide diffusion capacity; VTR: peak velocity of tricuspid regurgitation; FEV1: forced expiratory volume in 1 s. NA: not available.