Case 1
A 9 year-old boy underwent surgical repair of supracardiac TAPVC when he was 2 months old and weighed 2.2 Kg. [Figure1]
During the operation, the vertical vein was ligated. One year after the surgical repair, we performed follow up diagnostic catheterization that showed obvious azygos vein enlargement. Contrast enhanced CT was performed, and it showed a veno-venous shunt (VV shunt) that originated proximal to the ligated vertical vein and drained into the superior vena cava through the accessary hemiazygos vein-azygos vein. [Figure2]
These findings showed there was a second vertical vein causing TAPVC. Therefore, we corrected the diagnosis from a supracardiac TAPVC to a double drainage of TAPVC, a rare variant of the mixed type. The left-right shunting, which occurred as a result of this development, did not provoke right heart volume overload, and the general condition of the patient was good. His right heart function is under close monitoring in outpatient.