title: Prenatally diagnosed severe CDH: mortality and morbidity remain high.
abstract: OBJECTIVE
  This study sought to evaluate prenatal markers' ability to predict severe congenital diaphragmatic hernia(CDH) and assess this subgroup's morbidity and mortality.
  
  
  METHODS
  A retrospective review was performed between 2006 and 2014. Prenatal criteria for severe CDH included: liver herniation, lung-to-head ratio (LHR) <1 on prenatal ultrasound and/or observed-to-expected LHR (o/eLHR) <25%, and/or observed-to-expected total lung volume (o/eTLV) <25% on fetal MRI. Postnatal characteristics included: mortality, ECMO utilization, patch closure, persistent suprasystemic pulmonary hypertension (PHtn), O2 requirement at discharge, and few ventilator-free days in the first 60. Statistics performed used unpaired t-test, p<0.05 significant.
  
  
  RESULTS
  Overall, 47.5%(29/61) of patients with prenatally diagnosed, isolated CDH met severe criteria. Mean LHR: 1.04±0.35, o/eLHR: 31±10% and o/eTLV: 20±7%. Distribution was 72% LCDH, 24% R-CDH. Overall survival: 38%. ECMO requirement: 92%. Patch rate: 91%. Mean ventilator-free days in 60: 7.1±14. Supplemental oxygen at discharge was required in 27%. In this prenatally diagnosed severe cohort, 58%(15/26) had persistent PHtn post-ECMO requiring inhaled nitric oxide±epoprostenol. Comparing patients with and without PHtn: mean ECMO duration 18±10 days versus 9±7 days (p=0.01) and survival 20% versus 72% (p=0.006).
  
  
  CONCLUSIONS
  A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI. Electronic address: meganac@med.umich.edu.
authors.forename: Megan A
authors.initials: MA
authors.lastname: Coughlin
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Nicole L
authors.initials: NL
authors.lastname: Werner
authors.affiliation: Division of Pediatric Cardiology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Robert
authors.initials: R
authors.lastname: Gajarski
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Samir
authors.initials: S
authors.lastname: Gadepalli
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Ronald
authors.initials: R
authors.lastname: Hirschl
authors.affiliation: Division of Neonatology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: John
authors.initials: J
authors.lastname: Barks
authors.affiliation: Division of Maternal Fetal Medicine, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Marjorie C
authors.initials: MC
authors.lastname: Treadwell
authors.affiliation: Division of Pediatric Radiology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Maria
authors.initials: M
authors.lastname: Ladino-Torres
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: Jeannie
authors.initials: J
authors.lastname: Kreutzman
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
authors.forename: George B
authors.initials: GB
authors.lastname: Mychaliska
mesh_terms: D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis* / Q000473:pathology; D006257:Head / Q000000981:diagnostic imaging* / Q000196:embryology / Q000473:pathology; D006547:Hernia / Q000151:congenital / Q000175:diagnosis; D065630:Hernias, Diaphragmatic, Congenital / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis; D007231:Infant, Newborn; D008107:Liver Diseases / Q000151:congenital / Q000175:diagnosis; D008168:Lung / Q000000981:diagnostic imaging* / Q000196:embryology / Q000473:pathology; D008176:Lung Volume Measurements; D008279:Magnetic Resonance Imaging; D008297:Male; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D016216:Ultrasonography, Prenatal
keywords: Congenital diaphragmatic hernia; Fetal MRI; Lung to head ratio; Postnatal outcome; Prenatal diagnosis