Prenatally diagnosed severe CDH: mortality and morbidity remain high

Abstract Purpose 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). Conclusion A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.