Failure of Marfan anatomic criteria to predict risk of aortic dissection in Turner syndrome: necessity of specific adjusted risk thresholds.

ABSTRACT

Patients with Turner syndrome are prompt to develop spontaneous acute aortic dissection following insidious aortic dilatation, with abnormal cardiovascular anatomy and consequently require specific guidelines for regular surveillance since they represent a subset of high-risk young patients. We report a rare and uncommon case of spontaneous acute aortic dissection in a 48-year old female patient with Turner syndrome who was not apparently eligible for a prophylactic surgery. A CT scan showed a Stanford type A aortic dissection and was urgently referred for surgical management. We operated on the patient under deep hypothermia (18°C) and circulatory arrest with a retrograde cerebroplegia as the primary entry tear was located in the arch. The postoperative course was uneventful and the patient was discharged at the eighth postoperative day. Following description of this case, special attention was paid to determine predisposing risk factors for aortic dissection to be specifically adjusted to TS patients.