Scimitar syndrome with an accessory diaphragm and an absent right superior vena cava

Abstract

An 8-year-old boy with scimitar syndrome, an accessory diaphragm and an absent right superior vena cava, underwent surgery on March 28, 1983. The scimitar vein was separated from an accessory diaphragm and cut just above the right diaphragm where the vein penetrated. The vein was re-implanted into the right lateral portion of the right atrium and a tunnel was made between the atrial septal defect created in the septum and the site of the implanted vein. The accessory diaphragm was not removed because of the lack of compression on the right lung. At cardiopulmonary bypass, venous cannulae were inserted into the persistent left superior vena cava and inferior vena cava. Because of the absence of the right superior vena cava, the right atrium was not fixed by both cavae so that there was difficulty in intracardiac maneuvers. The patient is doing well 32 months after this treatment.