Prenatally diagnosed severe CDH: mortality and morbidity remain high

PurposeThis study sought to evaluate prenatal markers’ ability to predict severe congenital diaphragmatic hernia(CDH) and assess this subgroup’s morbidity and mortality.MethodsA 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.ResultsOverall, 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).ConclusionA combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.