Purpose: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency.
Methods: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30.
Results: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p=0.02), but not OELHR (p=0.12) or PPLV (p=0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p=0.03, p=0.02, p=0.01), ECMO (p<0.01, p<0.01, p=0.03), and ventilator dependency (p<0.01, p<0.01, p=0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p=0.04), ECMO (p=0.03), and ventilator dependency (p=0.02), while LHR was associated with ECMO (p=0.01) and ventilator dependency (p=0.02) but not mortality (p=0.06).
Conclusion: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes.
Keywords: Congenital diaphragmatic hernia; Extracorporeal membrane oxygenation; Magnetic resonance imaging; Prenatal diagnosis; Prenatal ultrasonography; Pulmonary hypoplasia.
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