%0 Journal Article
%T Prenatally diagnosed severe CDH: mortality and morbidity remain high.
%A Coughlin MA
%A Werner NL
%A Gajarski R
%A Gadepalli S
%A Hirschl R
%A Barks J
%A Treadwell MC
%A Ladino-Torres M
%A Kreutzman J
%A Mychaliska GB
%J J Pediatr Surg
%V 51
%N 7
%D Jul 2016
%M 26655216
%F 2.549
%R 10.1016/j.jpedsurg.2015.10.082
%X <B>OBJECTIVE: </B>This study sought to evaluate prenatal markers' ability to predict severe congenital diaphragmatic hernia(CDH) and assess this subgroup's morbidity and mortality.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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).<BR><B>CONCLUSIONS: </B>A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.