Fetal biophysical predictors of pulmonary hypertension severity, management, and treatment in infants born with congenital diaphragmatic hernia

Background: Pulmonary hypertension (PHTN) causes significant morbidity and mortality in patients with congenital diaphragmatic hernia (CDH). Currently, there is no routinely obtained prenatal prognostic marker to reliably predict postnatal CDH-associated PHTN severity. Methods: The CDH Study Group (CDHSG) registry was queried for infants born from 2015 to 2021 with a graded (1-4) PHTN diagnosis. Fetal observed-to-expected lung volume to head circumference ratio (o/e LHR), percent predicted lung volume (PPLV), and total lung volume (TLV) were classified by severity. Results: Of 4056 patients, 1047 and 785 infants had prenatal ultrasound or magnetic resonance imaging, respectively. Both moderate and severe o/e LHR were associated with increased odds of postnatal development of moderate (OR 2.913) and severe PHTN (OR 4.924). Conclusions: In infants with CDH, prenatal predictor severity was associated with higher severity of PHTN and increased ECLS usage. Overall, patients with worse prenatal prognostic indicators were less likely to receive pulmonary vasodilator treatment.