Link: https://www.ncbi.nlm.nih.gov/pubmed/30442461

J Pediatr Surg. 2018 Oct 5. pii: S0022-3468(130657-2. doi: 10.1016/j.jpedsurg.2018.10.039. [Epub ahead of print]
Use of prostaglandin E1 to treat pulmonary hypertension in congenital diaphragmatic hernia.
Lawrence KM1, Berger K1, Herkert L1, Franciscovich C1, O'Dea CLH2, Waqar LN1, Partridge E1, Hanna BD3, Peranteau WH4, Avitabile CM3, Hopper RK5, Rintoul NE3, Hedrick HL6.
Author information
1
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
2
Department of Pediatrics, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.
3
Department of Pediatrics, Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania.
4
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA; Perelman School of Medicine at the University of Pennsylvania.
5
Department of Pediatrics, Lucille Packard Children's Hospital, Palo Alto, California.
6
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA; Perelman School of Medicine at the University of Pennsylvania. Electronic address: Hedrick@email.chop.edu.
Abstract
BACKGROUND/PURPOSE:
Prostaglandin E1 (PGE) has been used to maintain ductus arteriosus patency and unload the suprasystemic right ventricle (RV) in neonates with congenital diaphragmatic hernia (CDH) and severe pulmonary hypertension (PH). Here we evaluate the PH response in neonates with CDH and severe PH treated with PGE.

METHODS:
We performed a retrospective chart review of CDH infants treated at our center between 2011 and 2016. In a subset, PGE was initiated for echocardiographic evidence of severe PH, metabolic acidosis, or hypoxemia. To assess PH response, we evaluated laboratory data, including B-type natriuretic peptide (BNP) and echocardiograms before and after PGE treatment. Categorical and continuous data were analyzed with Fisher's exact tests and Mann-Whitney t-tests, respectively.

RESULTS:
Fifty-seven infants were treated with PGE a mean 17 ± 2 days. BNP levels declined after 1.4 ± 0.2 days of treatment and again after 5.2 ± 0.6 days. After 6 ± 0.8 days of treatment, echocardiographic estimates of severe PH by tricuspid regurgitation jet velocity, ductus arteriosus direction, and ventricular septum position also improved significantly. Treatment was not associated with postductal hypoxemia or systemic hypoperfusion.

CONCLUSIONS:
In patients with CDH and severe PH, PGE is well tolerated and associated with improved BNP and echocardiographic indices of PH, suggesting successful unloading of the RV.

TYPE OF STUDY:
Treatment study.

LEVEL OF EVIDENCE:
Level III.

Copyright © 2018. Published by Elsevier Inc.

KEYWORDS:
Congenital diaphragmatic hernia; Prostaglandin E1; Pulmonary hypertension

PMID: 30442461 DOI: 10.1016/j.jpedsurg.2018.10.039
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