Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism.

We report the prenatal diagnosis of total anomalous pulmonary venous connection to the portal system in a 20-week fetus with right atrial isomerism. The apex of the fetal heart pointed to the left, the fetal stomach was on the right, there was a common atrioventricular valve, the left ventricle was small and the abdominal aorta and inferior vena cava were on the left side; all these features were suggestive of right atrial isomerism. An anomalous vein was connected to the portal vein which ascended above the diaphragm and ended in a confluence of pulmonary veins, posterior to the common atrium. Color Doppler imaging helped confirm the diagnosis of total anomalous pulmonary venous connection. The prenatal findings were confirmed on autopsy.