Congenital Pulmonary Malformation Volume Ratio Research Articles

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions.

To assess the diagnostic performance of the fetal cardiac axis (CA) and/or cardiac position (CP) versus the congenital pulmonary malformation volume ratio (CVR) in predicting any and severe neonatal respiratory morbidity in fetal congenital lung lesions. This work was an 11-year retrospective cohort study. The sensitivity, specificity, positive predictive value, and negative predictive value of CA and/or CP assessment in prediction of respiratory morbidity were calculated before 24 weeks' gestation and between 24 and 32 weeks and compared to CVR cutoffs obtained from the literature. Fifty-three patients were included. CA and/or CP abnormalities were present in 45% and 38% of patients before 24 weeks and between 24 and 32 weeks and were significantly more common in left- versus right-sided lesions (60% versus 17%; P = .003). The sensitivity, specificity, positive predictive value, and negative predictive value of an abnormal CA and/or CP for any and severe respiratory morbidity were 0.67, 0.61, 0.33, and 0.86 and 0.8, 0.58, 0.17, and 0.97 before 24 weeks and 0.75, 0.73, 0.45, and 0.91 and 0.8, 0.67, 0.20, and 0.97 between 24 and 32 weeks, respectively. An abnormal CA and/or CP had higher sensitivity for any respiratory morbidity compared to the CVR at 0.5 and 0.8 cutoffs both before 24 weeks and between 24 and 32 weeks (P < .05). An abnormal CA and/or CP before 24 weeks and between 24 and 32 weeks has higher sensitivity for the detection of any respiratory morbidity at birth compared to the CVR at both 0.5 and 0.8 cutoffs. A normal CA and CP have a high negative predictive value for excluding any respiratory morbidity at birth both before 24 weeks and between 24 and 32 weeks.

Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations.

Congenital pulmonary malformations (CPM) are mostly recognized on prenatal ultrasound scans. In a minority of cases, they may impair breathing at birth. The factors predictive of neonatal respiratory distress are not well defined, but an understanding of these factors is essential for decisions concerning the need for the delivery to take place in a tertiary care center. The aim of this study was to identify potential predictors of respiratory distress in neonates with CPM. We selected cases of prenatal diagnosis of hyperechoic and/or cystic lung lesions from RespiRare, the French prospective multicenter registry for liveborn children with rare respiratory diseases (2008-2013). Prenatal parameters were correlated with neonatal respiratory outcome. Data were analyzed for 89 children, 22 (25%) of whom had abnormal breathing at birth. Severe respiratory distress, requiring oxygen supplementation or ventilatory support, was observed in 12 neonates (13%). Respiratory distress at birth was significantly associated with the following prenatal parameters: mediastinal shift (P = .0003), polyhydramnios (P = .05), ascites (P = .0005), maximum prenatal malformation area (P = .001), and maximum congenital pulmonary malformation volume ratio (CVR) (P = .001). Severe respiratory distress, requiring oxygen at birth, was best predicted by polyhydramnios, ascites, or a CVR >0.84. CVR >0.84, polyhydramnios, and ascites increased the risk of respiratory complications at birth in fetuses with CPM, and especially of severe respiratory distress, requiring oxygen supplementation or more intensive intervention. In such situations, the delivery should take place in a tertiary care center.