Magnetic Resonance Imaging Assessment of Pulmonary Vascularity in Infants with Congenital Diaphragmatic Hernia: A Novel Tool for Direct Assessment of Severity of Pulmonary Hypertension and Hypoplasia

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 nonpulmonary birth defects served as controls (n = 5). Semiautomatic 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 = .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 = .01). Ipsilateral vascular density on MRI in infants with CDH significantly correlated with the prenatal pulmonary hypertensive index (P = .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 = .02, Spearman R = −0.47), use of epoprostenol for acute pulmonary hypertension (PH) (0.14 mm³/mm³ vs 0.20 mm³/mm³, P = .005), and use of sildenafil for chronic PH (0.15 mm³/mm³ vs 0.19 mm³/mm³, P = .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.