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Objective To evaluate the neonatal outcome of fetuses with isolated right‐sided congenital diaphragmatic hernia (iRCDH) based on prenatal severity indicators and antenatal management. Methods This was a retrospective review of prospectively collected data on consecutive cases diagnosed with iRCDH before 30 weeks' gestation in four fetal therapy centers, between January 2008 and December 2018. Data on prenatal severity assessment, antenatal management and perinatal outcome were retrieved. Univariate and multivariate logistic regression analysis were used to identify predictors of survival at discharge and early neonatal morbidity. Results Of 265 patients assessed during the study period, we excluded 40 (15%) who underwent termination of pregnancy, two cases of unexplained fetal death, two that were lost to follow‐up, one for which antenatal assessment of lung hypoplasia was not available and six cases which were found to have major associated anomalies or syndromes after birth. Of the 214 fetuses with iRCDH included in the neonatal outcome analysis, 86 were managed expectantly during pregnancy and 128 underwent fetal endoscopic tracheal occlusion (FETO) with a balloon. In the expectant‐management group, lung size measured by ultrasound or by magnetic resonance imaging was the only independent predictor of survival (observed‐to‐expected lung‐to‐head ratio (o/e‐LHR) odds ratio (OR), 1.06 (95% CI, 1.02–1.11); P = 0.003). Until now, stratification for severe lung hypoplasia has been based on an o/e‐LHR cut‐off of 45%. In cases managed expectantly, the survival rate was 15% (4/27) in those with o/e‐LHR ≤ 45% and 61% (36/59) for o/e‐LHR > 45% (P = 0.001). However, the best o/e‐LHR cut‐off for the prediction of survival at discharge was 50%, with a sensitivity of 78% and specificity of 72%. In the expectantly managed group, survivors with severe pulmonary hypoplasia stayed longer in the neonatal intensive care unit than did those with mildly hypoplastic lungs. In fetuses with an o/e‐LHR ≤ 45% treated with FETO, survival rate was higher than in those with similar lung size managed expectantly (49/120 (41%) vs 4/27 (15%); P = 0.014), despite higher prematurity rates (gestational age at birth: 34.4 ± 2.7 weeks vs 36.8 ± 3.0 weeks; P < 0.0001). In fetuses treated with FETO, gestational age at birth was the only predictor of survival (OR, 1.25 (95% CI, 1.04–1.50); P = 0.02). Conclusions Antenatal measurement of lung size can predict survival in iRCDH. In fetuses with severe lung hypoplasia, FETO was associated with a significant increase in survival without an associated increase in neonatal morbidity. © 2020 International Society of Ultrasound in Obstetrics and Gynecology

Objectives In fetuses with isolated left‐sided congenital diaphragmatic hernia (LCDH), prenatal detection of severe pulmonary hypoplasia is important, as fetal therapy can improve survival. Cases with mild or moderate lung hypoplasia still carry a considerable risk of mortality and morbidity, but there has been less interest in the accurate prediction of outcome in these cases. In this study of fetuses with mild or moderate isolated LCDH, we aimed to investigate: (1) the association between intrapulmonary artery (IPA) Doppler findings and mortality at discharge; (2) whether adding IPA Doppler findings improves the prediction of mortality based on lung size and liver herniation; and (3) the association between IPA Doppler findings and early neonatal morbidity. Methods This was a retrospective study of all consecutive fetuses assessed at the BCNatal and UZ Leuven hospitals between 2008 and 2020 with a prenatal diagnosis of isolated, non‐severe LCDH, defined as observed‐to‐expected lung‐to‐head ratio (o/e‐LHR) > 25%, that were managed expectantly during pregnancy followed by standardized neonatal management. An additional inclusion criterion was the availability of IPA Doppler measurements. The primary outcome was the association between IPA Doppler findings and mortality at discharge. Other predictors included o/e‐LHR, liver herniation and gestational age at birth. Secondary outcomes were the association between IPA Doppler findings and the presence of pulmonary hypertension (PHT), need for supplemental oxygen at discharge and need for extracorporeal membrane oxygenation. IPA pulsatility index (PI) values were converted into Z‐scores. Logistic regression analysis was performed to investigate the associations between predictor variables and outcome, and the best model was chosen based on the Nagelkerke R2. Results Observations for 70 non‐severe LCDH cases were available. Fifty‐four (77%) fetuses survived until discharge. On logistic regression analysis, higher IPA‐PI was associated with an increased risk of mortality (odds ratio (OR), 3.96 (95% CI, 1.62–9.70)), independently of o/e‐LHR (OR, 0.87 (95% CI, 0.79–0.97)). An IPA‐PI Z‐score cut‐off of 1.8 predicted mortality with a detection rate of 69% and specificity of 93%. Adding IPA‐PI to o/e‐LHR improved significantly the model's performance (Nagelkerke R2, 46% for o/e‐LHR + IPA‐PI vs 28% for o/e‐LHR (P < 0.002)), with a detection rate of 81% at a 10% false‐positive rate. IPA‐PI was associated with PHT (OR, 2.20 (95% CI, 1.01–4.59)) and need for oxygen supplementation at discharge (OR, 1.90 (95% CI, 1.10–3.40)), independently of lung size. Conclusions In fetuses with mild or moderate LCDH, IPA‐PI was associated with mortality and morbidity, independently of lung size. A model combining o/e‐LHR with IPA‐PI identified up to four in five cases that eventually died, despite being considered to have non‐severe pulmonary hypoplasia. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Objective To determine the prevalence of pulmonary hypertension (PAH) in left‐sided congenital diaphragmatic hernia (CDH); how we could predict it; and how PAH contributed to the model for mortality prediction. Study Design Retrospective analysis in three European centers. The primary outcome was the presence of PAH on postnatal day (d) 1, 7, and at discharge. Studied predictors of PAH were: observed/expected‐lung/head‐ratio (o/e LHR), liver‐herniation, fetoscopic endoluminal tracheal occlusion (FETO), and gestational age (GA) at delivery. The combined effect of pre‐ and postnatal variables on mortality was modeled by Cox regression. Results Of the 197 neonates, 56 (28.4%) died. At d1, 67.5% (133/197) had PAH and 61.9% (101/163) by d7. Overall, 6.4% (9/141) had PAH at discharge. At d1, o/e LHR (odds ratio (OR) 0.96) and FETO (OR 2.99) independently correlated to PAH (areas under the curve [AUC]: 0.74). At d7, PAH significantly correlated only with the use of FETO (OR 3.9; AUC: 0.65). None were significant for PAH at discharge. Combining the occurrence of PAH with antenatal biomarkers improved mortality prediction (p = 0.02), in a model including o/e LHR (HR: 0.94), FETO (HR: 0.35), liver herniation (HR: 16.78), and PAH (HR: 15.95). Conclusions Antenatal prediction of PAH was only moderate. The postnatal occurrence of PAH further increases the risk of death. Whereas this may be used to counsel parents in the postnatal period, our study demonstrates there is a need to find more accurate antenatal predictors for PAH.