Delayed aortic arch deformity associated with a frozen elephant trunk after total arch replacement

Abstract We report a case of progressive deformity of the native distal aortic arch at the level of the tip of a frozen elephant trunk (FET), which developed 5 years after total arch replacement with FET (TAR-FET) for acute Stanford type A aortic dissection. This deformity arose in the late postoperative period and progressed rapidly over the following 6 months without any change in aortic diameter, and no clinical symptoms—including upper-to-lower extremity blood pressure discrepancy—were observed throughout this course. The patient ultimately required re-total arch replacement with FET extension, a concomitant Bentall procedure, and thoracic endovascular aortic repair (TEVAR), the latter performed emergently on postoperative day 1 after detachment of the new FET from the descending aortic wall produced a marked upper-to-lower extremity pressure gradient; this resulted in complete restoration of aortic morphology. This case demonstrates that morphological change of the aortic arch after FET can progress silently in the late postoperative period and may not be detected by diameter-based surveillance criteria. It highlights the importance of evaluating aortic morphology, in addition to diameter, and of selecting an adequately long distal landing zone extending beyond the arch curvature, during long-term surveillance and reintervention planning.