CHAPTER 12

### PATIENT STORY

A 28-year-old woman presented to her local rural hospital with acute-onset severe chest pain which came on at rest. There was no inciting physical or emotional stressor. She had a known diagnosis of Turner syndrome (XO) with prior documentation of a normal functioning bicuspid aortic valve. Her diagnosis of bicuspid aortic valve was based on a cardiac catheterization performed in early childhood and subsequent clinical examinations. She had no murmur to suggest aortic insufficiency or stenosis and had no prior cardiac imaging evaluations. She was seen routinely by a pediatric cardiologist every 2 years until the age of 18 years. She was subsequently lost to follow up.

At the time of her last routine pediatric cardiology visit 10 years ago she had been provided with instructions regarding the need for routine care and the possible need for cardiovascular surgery in the future but had not been instructed on where to seek care now that she had reached adult age. She assumed she could no longer be seen at a pediatric institution and did not successfully transition to an adult facility.

At the time of her presentation to the emergency room, she had a blood pressure (BP) in both arms of 160/80 mm Hg. A chest x-ray was performed which showed a widened mediastinum thus prompting a computed tomography (CT) angiogram which demonstrated a dilated ascending aorta measuring 3.6 cm yielding an indexed score of 2.4 cm/m2 (with a normal value for this patient population defined as <2.2 cm/m2).1 There was no dissection of the ascending aorta. She had a bovine arch wherein the first head and neck vessel gave rise to both carotid arteries.2 There was focal aneurysmal dilation of the distal aortic arch extending to the proximal portion of the left subclavian artery and proximal descending thoracic aorta. There was a focal dissection beginning in the distal aortic arch with propagation into the proximal left subclavian artery and into the descending thoracic aorta. The right subclavian artery was aberrant in origin arising from the descending thoracic aorta just distal to the left subclavian artery.

The patient was started on a labetalol infusion with an achieved goal systolic BP of 100 mm Hg. Attempts were made to transfer the patient to an appropriate medical facility for further management. In light of the limited nature of the dissection, the lack of ascending aortic involvement, and a maximal aortic diameter less than established surgical criteria, an attempt at conservative management was attempted.3 Despite optimal blood pressure control, serial scans over a period of 4 days demonstrated progressive dilation of the involved aortic segment and thus the decision was made to proceed with intervention. A hybrid procedure approach was undertaken. Bilateral carotid artery to subclavian artery bypass grafts were first placed to establish flow to both subclavian vessels. This was then followed by placement of a covered stent in the distal ...

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