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Heart Transplantation With Repair Of Total Anomalous Pulmonary Venous Repair In A Patient With Heterotaxy
Carlos M. Mery, Neil M. Venardos, Chesney Castleberry.
Dell Medical School at The University of Texas at Austin / Dell Children's Medical Center, Austin, TX, USA.

Cardiac transplantation in the setting of heterotaxy frequently requires adjusting the surgical technique.We present the case of a 16-year-old male with heterotaxy, bilateral superior vena cavas (SVC), total anomalous pulmonary venous return (TAPVR) to the left SVC, a separate hepatic vein entrance into the atrium, right-ventricular-dominant atrioventricular septal defect, transposed great vessels, a hypoplastic left ventricle, and discontinuous pulmonary arteries (PAs). He had had multiple procedures including PA reconstruction and Sano conduit, bilateral bidirectional Glenns, and an extracardiac fenestrated Fontan, among others. The pulmonary venous confluence had been left draining into a vertical vein that traveled behind the bronchus and drained through the proximal left SVC into the atrium. He developed significant ventricular dysfunction and atrioventricular valve regurgitation requiring in-hospital admission and continuous milrinone. He was put forward for transplant.At the time of cardiac transplantation, the vertical vein was divided as part of the cardiectomy. A large opening was created on the posterior venous confluence and a sutureless repair of the TAPVR was performed by suturing the donor left atrial cuff around the pulmonary venous confluence. The vertical vein was tied off. The Fontan was taken down, the PAs reconstructed, the IVC and separate hepatic vein were anastomosed together to the donor IVC, and the left Glenn was left in place.The patient did well postoperatively and is now 2 years after transplantation with no pulmonary venous obstruction, normal ventricular function, and no rejection.


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