[A modified Fontan operation in the presence of a supracardiac total anomalous pulmonary venous connection]

We report a case of 2-year-old girl with asplenia syndrome who successfully underwent modified Fontan procedure and concomitant repair of supracardiac total anomalous pulmonary venous connection (TAPVC). The preoperative diagnosis included a common atrioventricular canal (type C), a double outlet right ventricle, a common atrium, common atrioventricular valve regurgitation, pulmonary stenosis, and a bilateral superior vena cava (SVC). Cardiac catheterization revealed a Qp/Qs of 1.3, mean PA pressure of 16 mmHg and an Rp of 1.3. The TAPVC drained to left SVC (LSVC) at a position proximal to the hemiazygos vein with an ostium of 5 mm in diameter. The LSVC was divided distal to its connection to the common pulmonary vein (CPV). The TAPVC ostium was cut back into the CPV, then it was anastmosed with posterior aspect of the atrial wall in an effort to provide a wide anastomosis. The postoperative course was uneventful and the patient was discharged from hospital on the 35th postoperative day.