Posterolateral Tunnel Fontan, Bilateral Classic Glenn Centralization, and Right Upper Pulmonary Vein Translocation

The patient was a 4-year, 3-month-old girl with a hypoplastic left heart syndrome (mitral atresia/aortic atresia), anomalous left coronary artery from the pulmonary artery, partial anomalous pulmonary venous connection (right upper pulmonary vein to right superior vena cava), and left superior vena cava to coronary sinus. On day of life four, the patient went to the operating room for a Norwood procedure. At the time of the procedure, ALCAPA and PAPVC had not been identified. The Norwood was aborted secondary to multiple cases of ventricular fibrillation prior to and following the pericardiotomy. The patient was immediately transported to the catheterization lab for a hybrid procedure (bilateral pulmonary artery bands and ductus arteriosus stent placement), followed by an atrial septostomy five days later. During the atrial septostomy procedure, the anomalous right upper pulmonary vein to right superior vena cava was identified. Bilateral superior vena cava without a bridging vein was also identified. The patient progressed well and was discharged for the interstage period.

At five months of age, the patient presented for stage II palliation. Intraoperatively, the patient’s anomalous left coronary artery was identified as originating proximal to the left pulmonary artery band. The band allowed the left coronary artery to be exposed to systemic pressure during the interstage period. The patient then underwent a modified Norwood procedure (modified arch reconstruction with ductal stent removal), bilateral classic Glenn shunts (to avoid potential ALCAPA compression), and anomalous right upper pulmonary vein repair.

At four years, three months of age, patient underwent a pre-Fontan catheterization. Despite multiple modalities of imaging (2D and cross-sectional imaging with 3D reconstructions), exact Fontan strategy was unclear. Intraoperatively, the patient underwent a posterolateral tunnel Fontan, centralization of bilateral classic Glenn shunts, translocation of the right upper pulmonary vein, and right atrial reconstruction. Centralization of the bilateral classic Glenns was made possible by extensive dissection and opening of the posterior pericardium to allow space for the interposition graft to lay without compression of the left coronary artery. The right upper pulmonary vein was translocated to allow use of the right atrial free wall for posterolateral tunnel incorporation. The patient had a relatively uneventful postoperative course and is doing well as an outpatient.