Terlipressin as rescue therapy for refractory pulmonary hypertension in a neonate with a congenital diaphragmatic hernia

Abstract

We report the case of a 38-week gestational age neonate, with isolated congenital diaphragmatic hernia presenting with refractory persistent pulmonary hypertension, systemic hypotension, and hypoxemia, resistant to usual therapeutics. Arginine vasopressin is responsible for systemic vasoconstriction and decreases pulmonary hypertension. We theorized that terlipressin, its long-acting analogue, could have the same properties. We used terlipressin as rescue therapy after parental and local ethics committee acceptance. After a bolus of terlipressin 20 μg/kg and continuous infusion at a rate of 5 μg/kg per hour, blood oxygen saturation improved from 75% to 98%, oxygen requirements fell from fraction of inspired oxygen 100% to 40%, and mean arterial pressure rose from 28 to 46 mm Hg, allowing a decrease of vasopressor infusion. Terlipressin may be useful in the management of neonates with congenital diaphragmatic hernia and refractory pulmonary hypertension.