The Role of Fetal Echocardiography in Predicting Postnatal Pulmonary Venous Obstruction in Neonates with TAPVR: A Single Center Experience.

Total anomalous pulmonary venous return (TAPVR) is a high risk and rare cardiac malformation with a low prenatal detection rate and predicting obstruction in these cases is difficult. We sought to investigate fetal echocardiographic parameters associated with postnatal pulmonary venous obstruction (PPVO). We performed a retrospective review of 26 patients with TAPVR who had a fetal echocardiogram from 2010 to 2021. Blinded readers analyzed the fetal Doppler features of the pulmonary veins (PV) and vertical veins (VV) at final fetal assessment. Of the 26 patients, 17 (65%) were prenatally diagnosed and overall 10 (38.5%) developed PPVO. All patients had additional associated congenital heart disease. Compared to patients without PPVO, patients with PPVO had a lower VV variability index (VI) (0.51 (0.17-0.79) vs 1.43 (0.54-2.58); p = 0.003), a higher likelihood of having an abnormal VV or PV Doppler pattern (p = 0.042, p = 0.015), a higher VV minimum velocity (0.435 m/s (0.17-1.05) vs 0.14 m/s (- 0.16 to 0.35) p = 0.001) and a higher VV mean velocity (0.641 m/s (0.31-1.15) vs 0.321 m/s (0.19-0.5); p = 0.009). A fetal VV VI of ≤ 0.8 was associated with PPVO (100% sensitivity, 86% specificity). Our data show that some fetal PV and VV Doppler indices are significantly different between those that develop PPVO and those who do not, with the most significant difference seen in the VV variability index and VV minimum velocity. Prenatal predictors of PPVO have the potential to influence prenatal counseling, perinatal care, surgical timing, and outcomes associated with this critical diagnosis.