Table 1:
Summary of Outcomes After PH-Targeted Therapy for SAPH
| Author: | Year | Treatment | n | Major Outcomes | Adverse effects |
|---|---|---|---|---|---|
| SAPH: | |||||
| Preston et al.15 | 2001 | iNO | 8 | ↓ mPAP 18%, ↓ PVR 31%, CO 12% | Ø adverse events |
| Preston et al.15 | 2001 | IV EPO | 6 | Ø mPAP, ↓ PVR 25%, CO 25% | Ø adverse events |
| Fisher et al.67 | 2006 | IV EPO | 7 | ↓ mPAP 21%, ↓ PVR 45%, CO 44%, WHO class 1–2 | ↓ PaO2 in 3/7 with one death |
| Baughman et al.70 | 2009 | Inhaled iloprost | 15 | ↓ mPAP 15%, ↓ PVR 14%, 6MWD 12%, QOL | ↓ PaO2 in 2/15 (mild) |
| Milman et al.5 | 2008 | Sildenafil | 12 | ↓ mPAP 19%, ↓ PVR 48%, CO 36%, Ø 6MWD | Ø adverse events |
| Baughman et al.73 | 2013 | Bosentan in RCT | 35 | ↓ mPAP 11%, ↓ PVR 28%, Ø 6MWD, WHO class or QOL | Ø adverse events |
| Barnett et al.33 | 2009 | IV EPO, sildenafil, bosentan | 22 | ↓ mPAP 20%, ↓ PVR 39%, 6MWD | Ø adverse events |
| Judson et al.74 | 2011 | Ambrisentan | 21 | Ø 6MWD, DLCO, QOL or dyspnea scores | Increased edema and dyspnea |
PH: pulmonary hypertension; SAPH: sarcoidosis associated pulmonary hypertension; iNO: inhaled nitric oxide; IV EPO intravenous epoprostenolol; mPAP = mean pulmonary artery pressure; PVR: mean pulmonary vascular resistance; CO: cardiac output; 6MWD: 6 minute walk distance; PaO2: partial pressure of oxygen; QOL: quality of life; DLCO: diffusion capacity for carbon monoxide; RCT: randomized placebo controlled trial.