Abstract

replacement. The sparing of the native aortic valve had the added advantages of excellent hemodynamics and no requirement for anticoagulation. Because the ascending aorta and aortic arch were of upper normal caliber, surgical replacement was not absolutely required at the same time. Our rationale for concomitant replacement was 2-fold. First, it added negligible extra perioperative risk, given his young age and our extensive experience in aortic arch surgery. Second, the patient was at a much higher risk of type A dissection in the near future, given the natural history of LDS. In summary, the clinical observation from this case is that surgical management of the proximal thoracic aorta in patients with LDS should take into account not only the presenting aortic pathology but also the likely future aortic syndromes based on the natural history of this aggressive vasculopathy. The aortopathy seen in patients with LDS merits an aggressive surgical approach, given its life-threatening presentations.

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