Valve-Sparing Root Replacement in Marfan Syndrome with Acute Type A Aortic Dissection

Acute type A aortic dissection (TAAD) is an emergency condition that requires immediate surgical intervention. Marfan syndrome (MFS) is an inherited autosomal dominant disease with an incidence of approximately 1 per 5000 live births. The mean age at death for untreated patients with MFS is 32 years with aortic dissection, aortic rupture, or cardiac failure caused by mitral and aortic valve insufficiency (also called “regurgitation”) as the predominant cause of death in more than 90% of cases. There is an overall annual risk of 0.17% of aortic events and death occurring in patients with MFS; the risk is 4 times higher with an aortic diameter greater than 50 mm. The best surgical options for treating patients presenting with MFS, TAAD, and a normal aortic valve remain controversial. The modified Bentall procedure with a composite graft replacement of the aortic root has been used for decades with acceptable results. Alternatively, emerging techniques of aortic valve-sparing root replacement are increasingly being evaluated as a potential option. The focus of this chapter will be the techniques of aortic valve-sparing root replacement in patients with MFS presenting with TAAD and normal aortic valve morphology, as well as the early and long-term results of this operation based on the current literature.

Keywords: Aortic aneurysm, Aortic annulus, Aortic root replacement, Aortic valve, Aortic valve insufficiency, Aortic valve stenosis, Aortic valve-sparing root technique, Bentall procedure, Bicuspid aortic valve, Cardiopulmonary bypass, Composite valve graft, Coronary ostia, Cross clamp, Dacron graft, Marfan syndrome, Neo-aortic sinuses, Reimplantation technique, Remodeling technique, Reoperation, Transesophageal echocardiogram.