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.
