title: 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.
authors.affiliation: Réanimation Pédiatrique et Néonatale, Brûlés Pédiatriques, Pôle RAUC, Centre Hospitalo-Universitaire Nord, Marseille, France.
authors.forename: Lefteris
authors.initials: L
authors.lastname: Stathopoulos
authors.forename: Claire
authors.initials: C
authors.lastname: Nicaise
authors.forename: Fabrice
authors.initials: F
authors.lastname: Michel
authors.forename: Laurent
authors.initials: L
authors.lastname: Thomachot
authors.forename: Thierry
authors.initials: T
authors.lastname: Merrot
authors.forename: Pierre
authors.initials: P
authors.lastname: Lagier
authors.forename: Claude
authors.initials: C
authors.lastname: Martin
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D002423:Cause of Death; D003692:Delayed-Action Preparations; D004351:Drug Resistance; D017809:Fatal Outcome; D006548:Hernia, Diaphragmatic / Q000150:complications; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D007363:Intensive Care Units, Neonatal; D008297:Male; D012772:Shock, Septic / Q000401:mortality; D016896:Treatment Outcome; D014662:Vasoconstrictor Agents / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D003692:Delayed-Action Preparations; D014662:Vasoconstrictor Agents