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

• Published in: The Journal of pediatrics Vol. 239; pp. 89 - 94
• Main Authors: Mukthapuram, Shanmukha, Beebe, Jessica, Tkach, Jean A., Arya, Shreyas, Haberman, Beth, Peiro, Jose, Lim, Foong-Yen, Woods, Jason C., Kingma, Paul S.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.12.2021
• Subjects: Case-Control Studies; Female; Hernias, Diaphragmatic, Congenital - diagnostic imaging; Humans; Hypertension, Pulmonary - diagnostic imaging; Infant, Newborn; Longitudinal Studies; Lung Volume Measurements - methods; Magnetic Resonance Imaging - methods; Male; pulmonary hypoplasia; Severity of Illness Index; vascular density; vascular growth; vascular volume; vascular growth; vascular density; vascular volume; PPHI; pulmonary hypoplasia; MRI; CDH; MMGI; PH; iNO; 2D TOF
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2021.07.059

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