Antenatal and postnatal lung and vascular anatomic and functional studies in congenital diaphragmatic hernia: Implications for clinical management

Abstract

Congenital diaphragmatic hernia is characterized by fetal and neonatal lung hypoplasia as well as vascular hypoplasia. Antenatal imaging studies have been performed that attempt to quantify the degree of hypoplasia and its impact on infant prognosis. Prenatal and perinatal growth of the lung and vasculature are interdependent and their continued coordinated growth is critical for survival after birth in this patient population. Lung protection strategies appear to improve survival in newborns with diaphragmatic hernia, but a subset of infants remain who demonstrate sufficiently severe lung hypoplasia that we are unable to provide support long-term after birth. Fetal intervention is a strategy designed to enhance fetal lung growth towards improving survival in this most severely affected group, though other therapies to enhance postnatal lung and vascular growth should be concurrently investigated. However, any of these interventions will require careful selection of those infants at risk for poor outcome and thorough follow up, since long-term morbidity is significant in children with diaphragmatic hernia.

Fetal ultrasound markers of severity predict resolution of pulmonary hypertension in congenital diaphragmatic hernia

“…The technique consistently used and reported at UCSF uses the aorta and lateral rib as landmarks for the lateral measurement, with the orthogonal anterior-posterior (AP) diameter measured from the cardiac atria to the posterior rib, then dividing by the head circumference. 15,26 All analyses were done first including scans from 22 through 27 weeks’ GA 10,11 and then expanding to 20 to 29 weeks’ GA. As results did not differ when using these two inclusion criteria, data from the broader range of ages are presented to increase the information provided in our analyses. Liver position was gathered from fetal ultrasound reports, and verified by operative report from CDH repair, if surgery was performed.…”

Management of congenital diaphragmatic hernia

“…Prompted by the observation that congenital laryngeal atresia was associated with enlarged hyperplastic lungs, Wilson et al [92] showed that rapid pulmonary growth could be obtained by occluding the fetal trachea of the hypoplastic lung by, preventing the normal efflux of intraluminal lung liquid [93,94]. FETO accelerates fetal lung growth [95], with increased alveolar and capillary growth, and remodelling of pulmonary arterioles [93,94].…”

Plasma vascular endothelial growth factor A and placental growth factor: novel biomarkers of pulmonary hypertension in congenital diaphragmatic hernia

“…Pulmonary vascular density is reduced, vessel walls overly muscularized, and vasoreactivity impaired, leading to marked elevation of pulmonary vascular resistance (PVR) (15). The resultant pulmonary hypertension (PH) and secondary cardiac dysfunction are both strongly associated with acute and longterm morbidity and mortality in CDH (10,24,39).…”