J Pediatr

. 2021 Jul 30;S0022-3476(21)00751-4. doi: 10.1016/j.jpeds.2021.07.059. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/34339729/

MRI Assessment of Pulmonary Vascularity in Infants With Congenital Diaphragmatic Hernia: A Novel Tool for Direct Assessment of Severity of Pulmonary Hypertension and Hypoplasia

Shanmukha Mukthapuram ¹, Jessica Beebe ², Jean A Tkach ³, Shreyas Arya ⁴, Beth Haberman ¹, Jose Peiro ⁵, Foong-Yen Lim ⁵, Jason C Woods ⁶, Paul S Kingma ⁷Affiliations expand

• PMID: 34339729
• DOI: 10.1016/j.jpeds.2021.07.059

Abstract

Objectives: To assess the feasibility of magnetic resonance imaging (MRI) for postnatal assessment of pulmonary vascularity in infants with congenital diaphragmatic hernia (CDH).

Study design: Infants with prenatally diagnosed CDH (n=24) received postnatal pulmonary MRI. Infants with non-pulmonary birth defects served as controls (n=5). Semi-automatic segmentation was performed to obtain total vascular volume using time of flight images to assess vascularity.

Results: Average vascular density (vascular volume/lung volume) in control infants was 0.23±0.06 mm³/mm³ compared with 0.18±0.06 mm³/mm³ in infants with CDH is (p=0.09). When stratified further based on CDH severity, the difference between control infants and moderate CDH group was statistically significant. (0.23 mm³/mm³ vs 0.15 mm³/mm³, p=0.01). Ipsilateral vascular density on MRI in infants with CDH significantly correlated with the Prenatal Pulmonary Hypertensive Index (p=0.0004, Spearman R=+0.87) and with number of days on mechanical ventilation (P = .04, Spearman R=-0.44), total days on inhaled nitric oxide (p=0.02), use of epoprostenol for acute PH (0.14 mm³/mm³ vs 0.20 mm³/mm³, p=0.005) and use of sildenafil for chronic PH (0.15 mm³/mm³ vs 0.19 mm³/mm³, p=0.03).

Conclusions: Our results suggest that postnatal pulmonary vascularity assessed by MRI strongly correlates with prenatal and postnatal markers of PH severity and that pulmonary vascularity may serve as a direct measure of pulmonary vascular hypoplasia in infants with CDH.

Copyright © 2021. Published by Elsevier Inc.