Marfan syndrome: diagnosis and replacement of the aortic arch utilizing retrograde cerebral perfusion

The patient with a diagnosis of Marfan syndrome often presents to the cardiac surgical team for repair of the various cardiac and vascular abnormalities associated with the disease. One of the most complicated presentations involves an aneurysmal dilatation of the ascending and transverse aortic arch with or without involvement of the great vessels. The aortic valve may also be affected resulting in valvular insufficiency. These patients are usually in their 20s or 30s and undertaking a major surgical repair of the aortic arch exposes them to a variety of complications, which can have devastating effects. This article will review the diagnosis and pathology of the Marfan syndrome and describe the application of retrograde cerebral perfusion to lessen the chance of neurological morbidity following surgical replacement of the ascending and transverse aortic arch.