Cardiac transplantation in situs inversus post-TAPVC repair

Cardiac transplantation in situs inversus with dextrocardia is rare and surgically complex, owing to the reconstruction of systemic and pulmonary venous pathways. We present a 21-year-old male diagnosed with complex cyanotic congenital heart disease with situs inversus, dextrocardia, mixed total anomalous pulmonary venous connection (TAPVC), and good ventricular function with single ventricle physiology. He underwent left superior vena cava (LSVC) to left pulmonary artery (LPA) bidirectional (BD) Glenn shunt, with mixed TAPVC repair at 3 years of age. At the age of 21 years, he developed severe restrictive myocardial dysfunction and was not suitable for Fontan completion surgery. Hence, he was considered for orthotopic cardiac transplantation. The donor pericardium and donor heart were harvested with the full length of the superior vena cava (SVC) and the innominate vein. The ascending aorta and the aortic arch were harvested to the maximum possible length. After recipient cardiectomy and taking down the Glenn shunt, the recipient's right atrium (RA) was constructed into a composite tunnel by suturing the retained right atrial cuff posteriorly with bovine pericardium anteriorly. The donor inferior vena cava (IVC) was anastomosed to this tunnel. The donor right SVC was anastomosed to the recipient LSVC using the donor aortic homograft as an interposition graft. The native pulmonary artery (PA) confluence was anastomosed to the donor PA using donor pericardium to augment the anterior and lateral parts of the conduit, followed by aortic anastomosis. Post-procedure, the patient performed well with acceptable hemodynamics, and the heart occupied a mesocardial position.