Key result
Inverted four-branched arch graft with FET achieves branch patency and good 3-year outcomes.
Why the study?
The coexistence of acute type A aortic dissection and situs inversus totalis is exceedingly rare, and no prior case in a non-pregnant adult had been treated with combined ascending aorta replacement, total arch replacement, and frozen elephant trunk implantation.
Case Report (n=1)
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Demonstrates the feasibility of an inverted four-branched graft technique for total arch replacement and frozen elephant trunk implantation to accommodate mirrored anatomy in patients with situs inversus totalis and acute type A aortic dissection.
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.
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He et al. (2026) conducted a case report in Situs inversus totalis with mirror-image dextrocardia and acute type A aortic dissection (n=1). Ascending aorta replacement plus total arch replacement and frozen elephant trunk implantation using an inverted four-branched graft was evaluated on Graft patency and clinical survival/condition. Ascending aorta and total arch replacement with an inverted 4-branched graft and FET yielded patent branches at 10 months and good clinical status at 3 years.
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