Abstract

Growing evidence indicates fetal endoscopic tracheal occlusion (FETO) improves perinatal survival in fetuses with severe congenital diaphragmatic hernia (CDH).This study evaluates the impact of FETO on the resolution of pulmonary hypertension (PH) in fetuses with isolated severe left or right-sided CDH. All fetuses evaluated for CDH between January 2004 and July 2017 at a single institution were included. The resolution of PH by 1 year of life was compared in a cohort of infants with severe CDH who underwent FETO to a non-FETO cohort. The non-FETO cohort included CDH neonates stratified by prenatal MRI severity indices, [observed to expected total lung volume (O/E TLV) and % liver herniation] into mild, moderate, and severe groups. Postnatal echocardiograms were used to determine presence of PH, defined as elevation of pulmonary arterial pressure > 2/3 systemic systolic pressure based on Doppler parameters of the ductus arteriosus, interventicular septal position, and/or peak tricuspid regurgitant jet velocity. Patients were included if they underwent at least one prenatal MRI, had serial echocardiograms (the first within 48 hours of life) and had no other major structural or chromosomal anomalies. ANOVA and Dunnett’s post hoc tests were used for comparisons. A total of 254 CDH cases were evaluated; 57% (n=145) had no major structural or chromosomal anomalies and 106 met inclusion criteria. The FETO cohort consisted of 18 severe CHD cases and the non-FETO cohort consisted of 35% mild (n=31), 36% moderate (n=32), 29% severe (n=25) CHD cases. The proportion of left-or right-sided CDH cases was not different among groups. All infants with severe CDH, with and without FETO, had evidence of neonatal PH (see Table). By 1 year of life, a higher proportion of infants with severe CDH who underwent FETO had resolution of PH compared to infants with severe CDH in the non-FETO cohort (66.7% vs. 28%; p: 0.021). Of note, this proportion was similar to that of the moderate cases in the non-FETO cohort (see Table). FETO was also associated with a reduced need for ECMO in severe CHD cases regardless of the site of the CDH lesion (p: 0.009). In severe CDH infants, FETO is associated with resolution of PH by 1 year of life akin to moderate CDH cases. FETO may induce changes in fetal lung architecture facilitating resolution of PH in severe CDH.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

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