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.