Literature DB >> 32951888

Predicting lethal pulmonary hypoplasia in congenital diaphragmatic hernia (CDH): Institutional experience combined with CDH registry outcomes.

Aimee G Kim1, Rodrigo Mon2, Monita Karmakar1, Pamela A Lally3, Ronald B Hirschl1, George B Mychaliska1, Erin E Perrone4.   

Abstract

BACKGROUND: The Severe Pulmonary Hypoplasia and Evaluation for Resuscitative Efforts (SPHERE) protocol was developed to attempt to identify CDH patients with likely lethal pulmonary hypoplasia. We present our experience with this protocol and utilize the CDH Registry to critically assess the protocol.
METHODS: SPHERE patients identified based on prenatal imaging (10/2009-1/2018) were offered ECMO if meeting postnatal physiologic criteria, while others received comfort measures. Within the CDH Registry, patients with suspected severe CDH were identified and separated into "passed" (lowest pCO2 ≤100) versus "failed" (lowest pCO2 >100) groups.
RESULTS: Of 23 SPHERE patients, 57% (13/23) passed criteria for ECMO and survival was 46% (6/13) in that cohort. Of 4912 patients in the CDH Registry, 265 met criteria. There was no difference in survival rates between those that "passed" (122/227; 54%) versus "failed" (18/38; 47%). However, the latter had longer ECMO runs and more required ventilator/ECMO support at 30 days. Amongst survivors, the "failed" group had longer hospital stays and more frequently required tube feeds at discharge.
CONCLUSIONS: The SPHERE protocol did not predict mortality in the CDH Registry. However, our data suggest resource utilization is significant when unable to reach pCO2 ≤100 despite resuscitation. Morbidity remains high in this group. LEVEL OF EVIDENCE: Level III ANNOTATION OF CHANGES: Institutional Review Board Approval at University of Michigan (HUM00031524 and HUM00044010) TYPE OF STUDY: Retrospective Review.
Copyright © 2020 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Congenital diaphragmatic hernia; Prenatal diagnosis; Pulmonary hypertension

Mesh:

Year:  2020        PMID: 32951888     DOI: 10.1016/j.jpedsurg.2020.08.010

Source DB:  PubMed          Journal:  J Pediatr Surg        ISSN: 0022-3468            Impact factor:   2.545


  2 in total

1.  Best pre-ductal PaO2 prior to extracorporeal membrane oxygenation as predictor of mortality in patients with congenital diaphragmatic hernia: a retrospective analysis of a Japanese database.

Authors:  Keita Terui; Taizo Furukawa; Kouji Nagata; Masahiro Hayakawa; Hiroomi Okuyama; Shoichiro Amari; Akiko Yokoi; Kouji Masumoto; Masaya Yamoto; Tadaharu Okazaki; Noboru Inamura; Katsuaki Toyoshima; Keiichi Uchida; Manabu Okawada; Yasunori Sato; Noriaki Usui
Journal:  Pediatr Surg Int       Date:  2021-09-06       Impact factor: 1.827

Review 2.  Congenital diaphragmatic hernia: a narrative review of controversies in neonatal management.

Authors:  Michelle J Yang; Katie W Russell; Bradley A Yoder; Stephen J Fenton
Journal:  Transl Pediatr       Date:  2021-05
  2 in total

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