Epoprostenol has been reported to improve hemodynamics and functional capacity in primary pulmonary hypertension. The effect of long-term epoprostenol on exercise capacity in portopulmonary hypertension (PoPHTN) has not been previously reported.
Charts of PoPHTN patients who were placed on therapy with epoprostenol were reviewed for demographics, epoprostenol dose, 6 minute walk distance at baseline, 1st follow up visit, and 3rd follow up visit, duration of epoprostenol therapy, and survival.
A total of 12 patients [age(yrs±SD) 51.4 ± 6.3, 6 males] with a history of PoPHTN and long-term epoprostenol therapy were identified. The median duration of therapy with epoprostenol was 97.5 weeks (interquartile range, 26 to 236 weeks). The dose of epoprostenol ranged from 6 ng/kg/min to 46 ng/kg/min during various followup periods. Eight out of 12 patients died (67%) over the follow-up period and the most common cause of death was hepatic failure (63%). In the 8 patients who died, the average duration of epoprostenol therapy to the time of death was 128± 71.79 weeks [duration(months±SD)]. The average 6 minute walk distance for all patients increased from a baseline of 1079.6±395.4 (ft±SD) to 1245.5±300.02 [(ft±SD) p=NS] at the first follow up visit and 1247.9 ± 375.19 [(ft±SD) p=NS] at the third follow up visit.CONCLUSIONS: Long-term infusion of eposprostenol may improve exercise capacity in PoPHTN as demonstrated by improved 6 minute walk distances. Patients with PoPHTN may be safely and effectively maintained on long-term epoprostenol therapy.
Continuous infusion of epoprostenol may be useful to increasing exercise capacity in PoPHTN and possibly severing as a bridge to transplantation.
M.M. Budev, None.