An unusual cause of chylothorax after Norwood stage one reconstruction.

We report an unusual cause of prolonged chylothorax drainage after Norwood stage one reconstruction. This 1-month-old girl's chylous drainage was refractory to medical treatment. Echocardiography revealed thrombosis of the superior vena cava. Upon sternotomy to remove the thrombus, we were surprised to find the ePTFE (expanded polytetrafluoroethylene) tube previously used for selective cerebral perfusion compressing the innominate vein and the pericardium-based aortic arch. We performed a superior vena cava thrombectomy and shortened the ePTFE tube. Her chylothorax subsided gradually. We suggest that external compression of the venous drainage system should be considered in patients with prolonged chylothorax drainage. Once medical treatment fails, early surgical exploration may be helpful to stop the chylothorax.