Situs Inversus Totalis with Mirror-Image Dextrocardia and Acute Type A Aortic Dissection Treated by Ascending Aorta Replacement Plus Total Arch Replacement and Frozen Elephant Trunk Using an Inverted Four-Branched Graft: A Case Report

Situs inversus totalis (SIT) is a rare congenital condition characterized by a complete mirror-image reversal of the thoracic and abdominal viscera, usually accompanied by dextrocardia. Acute type A aortic dissection (AAD) is a highly lethal cardiovascular emergency, and its coexistence with SIT is exceedingly rare. To the best of our knowledge, apart from a recently reported pregnant patient, no case of AAD in a non-pregnant adult with SIT has been treated with ascending aorta replacement combined with total arch replacement (TAR) and frozen elephant trunk (FET) implantation. In this report, we present such a case and share our surgical experience, particularly the inverted four-branched graft technique used to resolve the mismatch between the commercially available graft and the mirrored anatomy. The postoperative course was complicated by right renal malperfusion and stage 2 acute kidney injury. Overall renal function recovered with conservative management, although the right kidney subsequently became atrophic; otherwise, recovery was satisfactory. Computed tomographic angiography (CTA) at 10 months showed the graft and all reconstructed branches patent and the FET in position without endoleak, and the patient remained in good condition at the telephone follow-up approximately 3 years after surgery.