Efficacy of Treprostinil in Neonates with Congenital Diaphragmatic Hernia Complicated by Severe Pulmonary Arterial Hypertension

Abstract Background Congenital diaphragmatic hernia (CDH) is a life-threatening neonatal condition, and concurrent severe pulmonary arterial hypertension (PAH) is its leading cause of death. Objective To explore the perioperative efficacy of treprostinil in neonates with CDH and severe PAH. Methods This was a single-center retrospective observational study. Clinical data of 67 neonates with CDH and severe PAH were collected. All neonates received immediate endotracheal intubation, high-frequency oscillatory ventilation (HFOV), inhaled nitric oxide (iNO), and continuous intravenous treprostinil after birth. PAH severity was evaluated via preductal-postductal oxygen saturation (SiO₂) difference and echocardiographic parameters. Results Among the 67 neonates (40 males, 27 females; 49 left-sided CDH, 18 right-sided CDH), 24 hours after treprostinil treatment: peak tricuspid regurgitation velocity (TRV), tricuspid regurgitation pressure (TVP), preductal-postductal SiO₂ difference (△SiO₂), patent ductus arteriosus (PDA) diameter, and patent foramen ovale (PFO) diameter all decreased significantly (all p < 0.001), with reduced right-to-left shunting. Fifty neonates survived surgery, and their key indicators (△SiO₂, PDA, PFO, TRV, TVP) showed a downward trend within 2 weeks postoperatively. Seven neonates died within 4 days postoperatively due to refractory severe PAH and multiple organ dysfunction. Conclusion Early treprostinil combined with HFOV + iNO effectively reduces pulmonary artery pressure, improves right-to-left shunting, creates favorable conditions for CDH repair and postoperative recovery, and is well-tolerated in neonates with CDH and severe PAH.