Objective: TAPVC has deteriorate neonatal condition. It is usually accompanied by other cardiac anomalies. It is important to detect TAPVC prenatally and the associated pulmonary venous stenosis(PS) as well.

Methods: We reviewed fetal echocardiography from July 1996 to december 2008 at our center. We had 2895 congenital heart anomaly and suspected TAPVC in 73. We suspected TAPVC if pulmonary vein to left atrium was not definite or extrachamber was shown in 2D and color Doppler ultrasound.

Results: Among 73 fetuses, we could identify TAPVC in 50, 28 through postnatal echocardiography and 22 in autopsy record, 23 have not shown up again. Out of 28 deliveres, 13 were confirmed as TAPVC, 2 PAPVC, and 1 PS. Through autopsy, 9 out of 22 were confirmed as TAPVC and 1 PAPVC. The type of TAPVC showed 7 supracardiac, 3 cardiac, 2 infracardiac, and 1 mixed connection. Four deaths occurred due to postoperative acidosis, septic shock and sudden collapse of pulmonary vein. We suspected isolated TAPVC in 5 prenatally, which were proven as 4 TAPVC and 1 PS. The isolated TAPVCs are good state after surgery, but the fetus with PS has expired one day after birth.

Conclusions: In echocardiography prenatally, absence of typical pulmonary drain to left atrium or presence of extrachamber could provide simple clue for anomalous pulmonary venous connection. Indefinite extrachamber could not rule out anomalous pulmonary venous connection. On the contrary, in that case, pulmonary venous stenosis should be suspected, which seemed most important in prognosis.