J Pediatr Surg. 2020 Jan 28. pii: S0022-3468(20)30033-6. doi: 10.1016/j.jpedsurg.2020.01.008. [Epub ahead of print] https://www.ncbi.nlm.nih.gov/pubmed/32061366
Prostaglandin E1 in infants with congenital diaphragmatic hernia (CDH) and life-threatening pulmonary hypertension.
Le Duc K1, Mur S2, Sharma D3, Aubry E3, Recher M4, Rakza T2, Storme L2; Center for Rare Disease «Congenital Diaphragmatic Hernia».
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Abstract
BACKGROUND:
Suprasystemic pulmonary hypertension (PH) is highly predictive of pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH).
OBJECTIVES:
To report the effects and tolerability of Prostaglandin E1 (PGE1) in newborns with severe CDH and life-threatening PH.
METHODS:
Newborn infants with isolated CDH and life-threatening PH defined by an acute worsening of the cardiorespiratory function, and bidirectional or exclusive right-to-left shunting across the ductus arteriosus (DA) with an acceleration of the blood flow >1.5 m.s-1 assessed by Doppler echocardiography. Serial measurements of cardiorespiratory variables have been recorded before and after PGE1.
RESULTS:
18 infants (out of 102 in the cohort) were included in the study (gestational age: 39 ± 2 weeks). The median FiO2, and preductal and postductal SpO2 were 80% [50; 100], 91% [88; 95] and 86% [82; 91], respectively, before treatment. FiO2 decreased to 35% [30-40] (p = 0.001) at H6. Maximal blood flow velocities in the DA decreased after starting PGE1 from 2.2 m.s-1 [1.5-2.5] to 1 m.s-1 [0.55-1.2] (p < 0.001).
CONCLUSIONS:
PGE1 treatment improved oxygenation and circulatory function in newborn infants with severe CDH and life-threatening PH. Our data provide evidence that restrictive DA may result in suprasystemic pulmonary hypertension in CDH infants, and that PGE1 may improve cardiorespiratory failure through reopening of the DA.
TYPE OF STUDY:
Treatment study.
LEVEL OF EVIDENCE:
Level III.
Copyright © 2020 Elsevier Inc. All rights reserved.
KEYWORDS:
Congenital diaphragmatic hernia; Ductus arteriosus; Prostaglandin E1; Pulmonary hypertensionPMID: 32061366 DOI: 10.1016/j.jpedsurg.2020.01.008
