PROSTACYCLIN-ANALOG THERAPY FOR PULMONARY VASCULAR DYSFUNCTION DURING SEVERE ACUTE CHEST SYNDROME IN SICKLE CELL DISEASE.

Background: The purpose of this study is to evaluate the impact of parenteral epoprostenol in sickle cell adults patients presenting acute cor pulmonale (ACP) complicating severe acute chest syndrome (ACS). Methods: Retrospective single-center analysis of sickle cell patients in ICU with ACP complicating severe ACS receiving epoprostenol, in addition to usual care (prostacyclin group) or usual care alone (control group). Primary outcome: relative change in pulmonary artery systolic pressure over 1 day. Results: 45 patients were included in the study, including 21 who received usual care and 24 who received epoprostenol. There was a significant reduction in pulmonary artery systolic pressure (PASP) during the first 24 h in the prostacyclin group (from 69 [60-77] to 52 [42-61] mmHg, P < 0.001) but not in the control group (from 50 [40-56] to 50 [35-55] mmHg, P = 0.285). The median decrease in PASP was greater in the prostacyclin group than in the control group (-25% [-33;-13] vs. -2% [-8; 0], P < 0.001). Conclusion: Parenteral prostacyclin analog therapy is associated with a significant reduction in PASP in patients with SCD admitted in ICU with ACS-related ACP.