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Ann Thorac Surg 1997;64:1144-1145
© 1997 The Society of Thoracic Surgeons
DR DOUGLAS M. BEHRENDT (Iowa City, IA): Because dissection is rare in children and only 35% of the children in your study had aortic valve insufficiency, aortic root dilatation per se was the indication for operation in most of the patients.
You said that the average size of the aortic root was 6.2 cm, but the $64,000 question is: What is the cutoff point at which operation should be recommended? I presume this is age-related. Could you comment on that, please?
DR GILLINOV: There are insufficient data to determine a specific size at which the aortic root should be replaced in all children with Marfan's syndrome. In adults, we generally use 55 mm, but in children, we individualize the decision to replace the aortic root based on the size of the patient, the size of the aorta, the rate of dilatation of the aorta, and the aortic valve function. We do consult tables that give values for normal aortic diameter according to the age of the patient.
Almost all these children had an increase in the diameter of the aorta by 1 cm or more in the year preceding operation, and this also was an important factor in leading us to operation.
DR LARS G. SVENSSON (Burlington, MA): I enjoyed your paper very much. It raises a lot of questions for us, such as what to do with young patients with Marfan's syndrome and, as Dr Behrendt has just touched on, when to operate on them.
I wonder whether you have looked at the curves of aortic size versus height in children. I am aware of two curves that are available with statistical formulas for calculating the expected size of the aorta in children. Have you looked at, say, 2 standard deviations beyond that as an indication for operation? I agree with your point about rapid growth. It is obviously very important.
The other question I had is concerning any valve-preserving or valve reimplantation procedures that you perform. I realize from Dr Gott's previous report that this rarely has been done at Hopkins. I wonder whether you could comment on this, particularly in children and young women. Thank you.
DR GILLINOV: Thank you for your comments, Dr Svensson.
We do compare the sizes of the aortas in children with the Marfan syndrome to the expected aortic diameters for age group. In all cases, aortic roots in patients undergoing operation were more than 2 standard deviations greater than normal size. Again, though, the decision to replace the aortic root takes into consideration many factors, and, as you said, this includes the rate of dilatation of the aorta on serial imaging studies.
We have not performed any valve-sparing aortic root procedures in children with the Marfan syndrome. In our total surgical series of more than 200 adults, we have done two valve-preserving procedures. In a larger, multi-institutional series of 603 adult patients with Marfan's syndrome undergoing cardiac operations, 52 patients had valve-sparing aortic root replacement.
Follow-up in these patients is short, and 1 patient has required reoperation for recurrent aortic insufficiency. We recommend caution in the application of valve-preserving procedures to patients with the Marfan syndrome because biochemical and histologic studies demonstrate extensive disarray of the connective tissue in their valve leaflets, and we are concerned about the durability of valve preservation in these patients. Nevertheless, certain young patients, particularly young women, might be candidates for this type of procedure.
DR ANDREW D. COCHRANE (Melbourne, Australia): In these children or teenagers who have undergone ascending aortic replacement, what incidence of dissection have you seen in the descending aorta subsequently, during the teenage years, and how have you managed it? My experience has been fairly limited, but in 2 or 3 cases presenting subsequent to aortic operations, they have deteriorated and ruptured quite rapidly.
DR GILLINOV: As I noted, we have had only 1 patient in this series with dissection of the ascending aorta, and that was discovered at operation. Two patients have had second operations to treat distal dissections after initial aortic operations. One presented at age 6 years with dissection extending from the aortic arch to the abdominal aorta and underwent a standard two-stage elephant trunk repair.
A second patient underwent composite graft repair of the ascending aorta and subsequently had a new dissection of the arch and underwent arch replacement.
We had 1 patient who had rupture of the distal aorta during follow-up, but this actually was an aneurysm without dissection.
DR NICHOLAS T. KOUCHOUKOS (St. Louis, MO): I would like to get back to the question of valve-sparing operations. I think your observations are reasonable ones, but one must keep in mind that there are many patients with Marfan's syndrome who live into adulthood who have abnormal leaflets containing abnormal fibrillin, but who do not have aortic regurgitation or other problems related to their valve. So I am not sure that I would consider that to be the principal contraindication to a valve-sparing operation, particularly in an older child in whom one would like to avoid the use of anticoagulants.
Further, homografts will not be permanent substitutes for patients in this age group, and a repair may endure for as long as a homograft.
DR GILLINOV: We agree with your comments. Only about one third of adults with Marfan's syndrome eventually require cardiac operations. In the future, we may use valve-sparing techniques in selected patients, but until now we have been somewhat hesitant to do so.
Related Article
Ann. Thorac. Surg. 1997 64: 1140-1144.
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