title: Pulmonary hypertension in giant omphalocele infants.
abstract: BACKGROUND
  Pulmonary hypoplasia has been described in cases of giant omphalocele (GO), although pulmonary hypertension (PH) has not been extensively studied in this disorder. In the present study, we describe rates and severity of PH in GO survivors who underwent standardized prenatal and postnatal care at our institution.
  
  
  METHODS
  A retrospective chart review was performed for all patients in our pulmonary hypoplasia program with a diagnosis of GO. Statistical significance was calculated using Fisher's exact test and Mann-Whitney test (p<0.05).
  
  
  RESULTS
  Fifty-four patients with GO were studied, with PH diagnosed in twenty (37%). No significant differences in gender, gestational ages, birth weight, or Apgar scores were associated with PH. Patients diagnosed with PH were managed with interventions, including high frequency oscillatory ventilation, and nitric oxide. Nine patients required long-term pulmonary vasodilator therapy. PH was associated with increased length of hospital stay (p<0.001), duration of mechanical ventilation (p=0.008), and requirement for tracheostomy (p=0.0032). Overall survival was high (94%), with significantly increased mortality in GO patients with PH (p=0.0460). Prenatal imaging demonstrating herniation of the stomach into the defect was significantly associated with PH (p=0.0322), with a positive predictive value of 52%.
  
  
  CONCLUSIONS
  In this series, PH was observed in 37% of GO patients. PH represents a significant complication of GO, and management of pulmonary dysfunction is a critical consideration in improving clinical outcomes in these patients.
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: Emily A
authors.initials: EA
authors.lastname: Partridge
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, 19104.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Hanna
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: Howard B
authors.initials: HB
authors.lastname: Panitch
authors.affiliation: Division of Neonatology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: Natalie E
authors.initials: NE
authors.lastname: Rintoul
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: William H
authors.initials: WH
authors.lastname: Peranteau
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
authors.forename: N
authors.initials: N
authors.lastname: Scott Adzick
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States. Electronic address: hedrick@email.chop.edu.
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
mesh_terms: D005260:Female; D005865:Gestational Age; D006554:Hernia, Umbilical / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology*; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D010414:Pennsylvania / Q000453:epidemiology; D012189:Retrospective Studies; D012720:Severity of Illness Index
keywords: Abdominal wall defects; Pulmonary hypertension; Pulmonary hypoplasia; Reactive airway disease