In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia

We describe a rare case of right atrial isomerism, levocardia, right-side s tomach, obstructed supracardiac total anomalous pulmonary venous connection , double outlet right ventricle with complete atrioventricular septal defec t and absent spleen. From the pulmonary venous confluence behind the atrium an ascending as well as a descending vertical vein communicated with the s ystemic venous system in the supracardiac as well as the infracardiac posit ion. The pulsed and color Doppler examination of the individual pulmonary v eins as well as of the vertical vein helped in making the diagnosis of obst ructed total anomalous pulmonary venous connection. The diagnosis was made by fetal echocardiographic examination at 22 weeks of gestation and confirm ed on postnatal echocardiography, cardiac catheterization, and at surgery.