Prediction of Neonatal Respiratory Function and Pulmonary Hypertension in Fetuses with Isolated Congenital Diaphragmatic Hernia in the Fetal Endoscopic Tracleal Occlusion Era: A Single-Center Study

Abstract

Objective: To correlate prenatal indicators of pulmonary hypoplasia with neonatal lung function and pulmonary hypertension (PHT) in isolated congenital diaphragmatic hernia (iCDH). Materials and Methods: Prospective single-center study on 40 fetuses with iCDH either expectantly managed (n = 13) or undergoing tracheal occlusion (n = 27). Prenatal predictors included observed/expected lung-head ratio (O/E LHR), observed/expected total fetal lung volume, fetal pulmonary reactivity to maternal O₂ administration (Δpulsatility index, ΔPI) and liver-to-thorax ratio (LiTR) as measured in the second and third trimesters. Postnatal outcome measures included survival until discharge, best oxygenation index (OI) and alveolar-arterial oxygen gradient [D(A-a)O₂] in the first 24 h of life and the occurrence of PHT in the first 28 days of life. Results: Median gestational age (GA) at evaluations was 27.2 and 34.3 weeks. GA at delivery was 36.0 weeks, and overall survival was 55%. In the second trimester, measurement of lung size, LiTR and pulmonary reactivity were significantly related to survival and the best OI and D(A-a)O₂.The occurrence of PHT was better predicted by ΔPI and LiTR. Conclusions: O/E LHR, LiTR and vascular reactivity correlate with ventilatory parameters in the first 24 h of life. Occurrence of PHT at ≥28 days was best predicted by LiTR and ΔPI, but not by lung size.

Fetal Stomach Position Predicts Neonatal Outcomes in Isolated Left-Sided Congenital Diaphragmatic Hernia

“…Despite advances in antenatal diagnosis and perinatal treatment, significant morbidity and mortality persist, primarily due to pulmonary hypoplasia [2–5]. The degree of pulmonary hypoplasia can be assessed antenatally by various imaging approaches, including determination of liver position (intra-abdominal versus intrathoracic) and measurements of contralateral [the lung-to-head ratio (LHR) or observed-to-expected LHR], or total, lung size [3, 6–11]. However, clinical experience and published data suggest that there is variability in the assessment of these prognostic measurements, in particular LHR and liver position by sonography [7, 12–17].…”

Learning Curve for Intrapulmonary Artery Doppler in Fetuses with Congenital Diaphragmatic Hernia

“…As increasing studies incorporate the use of fetal lung Doppler to improve the prediction of survival [6,10,11] and neonatal morbidity [8,9] in CDH fetuses, examiners should perform a sufficient number of examinations before considering incorporating these Doppler parameters into their clinical practice. Taking into account the rarity of this congenital disease and the number of scans necessary to achieve competence, this training must be performed in referral units with experienced operators in order to accelerate the learning process.…”

“…Doppler evaluation of fetal lung circulation has been suggested as a surrogate of lung hypoplasia and intrapulmonary hypertension [2,3,4,5], allowing stratification of CDH cases with a high risk to develop severe neonatal pulmonary hypertension [6,7,8], neonatal morbidity [9] and mortality [6,10,11]. However, intrapulmonary artery Doppler evaluation is a demanding measurement in CDH fetuses due to the difficult observation and insonation of the intrapulmonay artery under a reduced lung size, and concerns exist whether the clinical application of such parameters could be influenced by the operator competences [5].…”

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“…Far less is known about the uncommon right‐sided CDH; however lung size, determined by US or MR, seems to be predictive, but the values for severity of hypoplasia used for left‐sided lesions cannot be interchanged with those used for right‐sided lesions . Studies dedicated to prenatal prediction of short‐term morbidity remain scarce, but it seems that the previous predictors, as well as vascular reactivity, can predict pulmonary hypertension and short‐term pulmonary morbidity . One very large French study came to the conclusion that gestational age at diagnosis can predict both mortality and morbidity …”