Steps of surgery:
The patient was taken up for a first stage bidirectional Glenn shunt. The chest was opened with a midline sternotomy. Both lobes of the thymus were excised. Cardiopulmonary bypass was established with aorta – innominate vein – right atrial cannulation. The ductus was transfixed with 2 sutures after division.
The MPA was then transected. The cardiac end was sutured off in 2 layers. MPA continued as RPA. The pulmonary end of MPA was dissected, mobilized fully and then separated from the aorta along its entire axis. The LPA was transected beyond the origin at its mid portion and an incision was made into the pulmonary end of the LPA extending upto the hilum so as to create wide mouth of opening. (Figures 1B, 1C) Thereafter the pulmonary end of MPA was turned down alongside the opened LPA and both stomas were anastomosed to each other with 6/0 continuous prolene sutures (Figure 1D, Figure 3).
Thereafter the bidirectional Glenn shunt was performed in standard fashion. She made an uneventful recovery with resting saturations on room air climbing to 85%. The LPA anatomy and the confluence were evaluated after 6 months using a CT angiogram and was found to be satisfactory (Figure 2B). She is awaiting rapid Fontan completion in view of her age.