Total anomalous pulmonary venous connection is a rare congenital heart defect that can be diagnosed prenatally and might be very important for perinatal management. In addition to other cardiac abnormalities (levocardia, situs inversus, small left ventricle, double outlet right ventricle, parallel great vessels, and hypoplastic aortic arch), total anomalous pulmonary venous connection of a subdiaphragmatic type was diagnosed during a prenatal echocardiography examination in the second half of pregnancy. Fetal echocardiography monitoring showed no signs of congestive heart failure. The neonate was born at 38 weeks of gestation at our tertiary center. Postnatal echocardiography revealed significant progression in neonatal hemodynamics, and early cardiac surgery, involving repair of the pulmonary veins, pulmonary artery banding, and aortic arch reconstruction, was performed with a good outcome. This case is an excellent example of the value of prenatal echocardiography.

TAPVC; total anomalous pulmonary venous connection; prenatal diagnosis; prenatal echocardiography; fetal echocardiography; fetal echo