231: Effect of longer extracorporeal life support and early treprostinil on survival in infants with severe congenital diaphragmatic hernia

BACKGROUND: Pulmonary hypertension (PH) and chronic respiratory failure in infants with severe congenital diaphragmatic hernia (CDH) contributes to poor survival. In 2021, we adopted a new strategy for neonates with severe CDH who received ECLS. This included repair on ECLS, use of bivalirudin, tolerance of longer ECLS runs and early initiation of treprostinil. Our goals were to come off ECLS on lower, lung-protective ventilator settings and with lower pulmonary artery pressures and improved right ventricular function. We hypothesized that this treatment strategy would lead to improved survival. METHODS: We reviewed infants with severe CDH (defined as a lung to head ratio (LHR) < 1 and observed to expected total fetal lung volume (O/E TFLV) < 25%) who also received ECLS and treprostinil at our center born between 2015-2023 and compared outcomes before and after initiation of this strategy. RESULTS: 15/136 infants met criteria for severe CDH and also received ECLS. Nine infants were treated prior to protocol implementation and 6 were treated with the new protocol. Survival among neonates with severe CDH was higher after introduction of our new protocol. At the time of decannulation from ECLS, there was a decrease in pulmonary hypertension and lower ventilator support was required in this later cohort. There was also both a higher average length of stay and an increase in average ECLS days in the new protocol group. CONCLUSIONS: Early treatment of PH with treprostinil, longer ECLS and strict lung-protective ventilation strategies may lead to increased survival in severe CDH.