Prediction of early emergent/urgent surgery for neonates with prenatal concern for congenital pulmunary airway malformation

Background/Purpose:
Neonates with severe cases of congenital pulmonary airway malformation (CPAM) may require early emergent/urgent surgery for cardiopulmonary compromise. The CPAM volume ratio (CVR) is often utilized for prenatal prognostication; however, the threshold that predicts early surgery is unknown. We hypothesized that a late gestation CVR threshold exists at which neonates with prenatally diagnosised CPAM would require early surgery.

Methods:
A single-center, retrospective cohort study was conducted of all neonates with a prenatal diagnosis of CPAM, born April 2011 to May 2019, who underwent surgery at a single tertiary referral center (TRC). Patients with suspected CPAM were manged through a multidisciplinary fetal diagnosis/therapy center. Demographics, clinical characteristics, and outcomes were collected. The latest gestation CVR prior to birth was utilized. Patients requiring early emergent/urgent surgery were compared to patients who underwent late non-emergent/urgent surgery. Univariate analysis and logistic regression receiver operating curve with area under the curve (AUC) analysis were performed.

Results:
Of 53 patients, 9 (17%) were in the early group and 44 (83%) in the late group. Patients were similar by sex, race, birth weight, and gestational age at birth (Image). Early patients had more comorbidities, were born at the TRC, and weighed less at time of surgery. Late patients had a median CVR of 0.43 (IQR 0.24-0.70) versus 1.45 (IQR 1.0-1.8) in early patients (p<0.001, Image). Early patients were more likely to stay intubated postoperatively and had a longer postoperative length of stay. Complications were similar between groups. A threshold CVR > 0.9 had the best prediction of need for early surgery (AUC: 0.89).

Conclusions:
Late gestation CVR appears to prognosticate the need for early emergent/urgent surgery for patients with prenatally diagnosed CPAM. CVR > 0.9 was associated with need for early surgery. Prospective studies are needed to validate late gestation CVR for prediction of emergent/urgent surgery.