Table 4.
No. | Original drug | Drug transitioned to | Publication year | Study design | PAH patients | Time | Measures | Outcome |
---|---|---|---|---|---|---|---|---|
Prospective | ||||||||
1 | Epoprostenol or treprostinil | Oral bosentan | 2004 / Suleman et al.38 | Prospective open-label | 23 | 12 months | Successful transition WHO FC 6MWD Echocardiogram | Only 9 patients (39%) were successfully transitioned to bosentan. In failure group, there was a trend toward higher doses and duration of PG therapy and higher PAP, though not statistically significant Half of failures in initial 8 weeks and half in subsequent 3–12 months |
2 | IV epoprostenol or IV treprostinil | Oral bosentan | 2006 / Steiner et al.39 | Prospective open-label | 22 | 17.7 ± 5.3 months | Change 6MWD - primary changes in prostanoid dosing, BORG score, FC, changes in PAH therapy, RVSP by echo | 10/22 patients were able to complete transition over 6 months (2–12 range) 3/10 late failures 41 m fall in 6MWD in transitioned patients at 1 year Borg unchanged Successful patients with lower RVSP, mPAP, better 6MWD, and FC and lower PG analogue doses vs. those who failed |
Retrospective | ||||||||
3 | SQ treprostinil | Oral sildenafil | 2007 / Keogh et al.21 | Retrospective | 14 | 3 months | Successful transition WHO FC 6MWD QoL Echocardiogram | 71% stayed on Sildenafil Improved QOL No other significant changes |
4 | IV epoprostenol | Oral bosentan or oral sildenafil | 2007 / Johnson et al.40 | Retrospective | 13 (2 failed epo wean and not transitioned) | 29.9 ± 11.6 months | RHC, FC, 6MWD | 9/13 unchanged FC 4/13 worse FC Normal pre-wean hemodynamics (mPAP < 30 mmHg or PVR < 4 WU) predicted successful transition 8 successful at end of study 1 death from SDH 4 worsened and restarted prostacyclin 6MWD unchanged All 4 who failed had abnormal hemodynamics pre-transition |
5 | Epoprostenol (17 patients) or treprostinil (4 patients) | Oral bosentan and/or sildenafil | 2008 / Diaz-Guzman et al.41 | Retrospective | 21 (15 successful; 6 failed transition) | 24.7 ± 13.6 months in ST 30 ± 5.6 in FT | 6MWD, FC, BNP | Successful in 15/21 (71.4%) Low doses of prostanoids, mPAP < 40 mmHg, 6MWD > 400 m, SLE-PH, and use of sildenafil could predict a higher likelihood of successful weaning |
6 | IV epoprostenol | Oral bosentan | 2009 / Safdar42 | Retrospective | 11 | 3 months | Successful transition Adverse events WHO FC 6MWD | 7/11 patients required resumption of infused prostanoid 57% remained stable for “substantial period of time” on oral therapy 2 discontinued due to abnormal LFTs |
7 | Parenteral epoprostenol or treprostinil | Oral ERA/PDE5i | 2013 / Escolar et al.43 | Retrospective | 22 | 60 months | Successful transition Adverse events WHO FC 6MWD NT-proBNP Hemodynamics | Successful in 50% Failure associated with: Age > 55 years, idiopathic PAH, combination therapy, abnormal hemodynamics (RAP > 5 mmHg, mPAP > 40 mmHg, PASP > 70 mmHg, PVR > 6.5 WU |
mPAP, mean pulmonary artery pressure; PG, prostaglandin; PASP, pulmonary artery systolic pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RVSP, right ventricular systolic pressure; SDH, subdural hematoma; WU, Wood units.