Cardiac Transplantation in Situs Inversus Totalis: A Surgical Challenge

Abstract

A 45-year-old male patient diagnosed with non-compacted right ventricle, situs inversus totalis and dilated cardiomyopathy presented a 6-month report of decompensated heart failure symptoms (fatigue on moderate exertion, dyspnea at rest, and paroxysmal nocturnal dyspnea). As he did not respond to clinical treatment, with dependence on inotropic drugs and a recurrent hospitalization history, he underwent bicaval orthotopic heart transplant with a normal heart donor. The implant was made with ¼ h counterclockwise rotation, bringing the left auricle to medial position and the right chambers to a posterior position (retroaortic superior vena cava and posterior inferior vena cava). The left ventricle was anteriorized; the isotopic pulmonary trunk and the aorta were both in end-to-end anastomosis. The patient evolved well and was discharged on the 28th postoperative day. Ten months after surgery, he was still hemodynamically stable and without any signs of rejection. There are few scientific reports of cardiac transplantation in patients with situs inversus totalis. The greatest difficulty of heart transplant in patients with situs inversus totalis is the reconstruction of the systemic and pulmonary venous pathways. Besides the alignment of the inferior vena cavas, seating the normal donor heart in dextrocardia allows a better accommodation of the organ in the recipient pericardial cavity; it avoids ventricular compression from the three-lobed left lung, which would simulate a tamponade. These were the reasons which led to the choice for this surgical technique in this case.