SURGICAL-TREATMENT OF AORTIC ROOT ANEURYSM RELATED TO MARFAN-SYNDROMEIN EARLY-CHILDHOOD

The prognosis of Marfan syndrome in both adult and pediatric patients is primarily related to the cardiovascular complications. In infantile Marfan syn drome, although involvement of the mitral valve is the mos t frequently encountered cardiovascular lesion, the aortic root can be more worrisome because of its excessive dilatation, leading to aortic insufficiency or dissection. If the role of elective surgery is relat ively well defined for adult patients, it is still debated during chil dhood. We report two patients, aged 22 months and 5 years, each presen ting an aortic root aneurysm related to Marfan syndrome, and each trea ted with the Bentall procedure without specific age-related mortality or morbidity. These two patients experienced normal growth and were fr ee of any complication for a follow-up period of 8 and 2 years, respec tively. More than an absolute value of the aortic root dimension, it i s the conjunction of the rate of progression of the aortic root dilata tion, the degree and the duration of the aortic valve regurgitation, a nd its resulting left ventricular dysfunction that must be taken into consideration in choosing the surgical option.