Marfan Syndrome Research - Genetics, Causes, Symptoms, Treatment

Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.


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When should the aortic arch be replaced in Marfan patients?

Bachet J, Larrazet F, Goudot B, Dreyfus G, Folliguet T, Laborde F, Guilmet D

Département de Pathologie Cardiaque, Institut Mutualiste Montsouris, Paris, France. jean.bachet@imm.fr

BACKGROUND: The purpose of this study was to assess the prevalence, indications, and results of aortic arch replacement in Marfan patients with and without acute dissection. METHODS: Between January 1993 and December 2005, our group performed 76 aortic replacements in 54 Marfan patients (mean age, 38.3 years), of whom 20 had already undergone one or two replacements of the thoracic aorta, and 3 required one late procedure each in other institutions. So, the 54 patients underwent a total of 100 aortic operations. Indication for initial surgery was elective aortic root replacement in 25 patients (46%), acute type A dissection in 19 (35%), acute type B dissection in 2 (4%), and chronic type B dissection in 8 (15%). Indication for reoperation was residual chronic dissection in the proximal aorta in 14 patients (36%), in the distal aorta in 22 (56%), and acute retrograde type A dissection in 3 (8%). RESULTS: At initial operation, the aortic arch was not involved in the 25 patients with aneurysm of the aortic root and was replaced in only 1 of the 19 patients with acute type A dissection (1/44 patients, 2.3%). At the second or third operation, the arch had to be replaced in 4 (16%) of 25 patients initially operated on for aortic root aneurysm, in 14 (73%) of 19 patients operated on for acute type A dissection, and in 3 (30%) of 10 patients with previous acute or chronic type B dissection. The difference between patients with initial elective aortic root replacement and patients with acute dissection was highly significant (p < 0.001). Overall in-hospital mortality was 13%. The risk of death was 9.6% per procedure. CONCLUSIONS: Aortic arch replacement in Marfan patients is not indicated during elective aortic root replacement. In contrast, the significant rate of aneurysmal dilatation of the aortic arch after surgery for acute type A dissection may be an incentive for a more aggressive approach toward the aortic arch during initial surgery.

Published 29 January 2007 in Ann Thorac Surg, 83(2): S774-9; discussion S785-90.
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Marfan Syndrome Research Today Archive:

Volume 1 (2005)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
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  Issue 7 (July)
  Issue 8 (August)
  Issue 9 (September)
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Volume 2 (2006)
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  Issue 3 (March)
  Issue 4 (April)
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Volume 3 (2007)
  Issue 1 (January)
  Issue 2 (February)
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  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
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  Issue 8 (August)
  Issue 9 (September)
  Issue 10 (October)
  Issue 11 (November)
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Volume 4 (2008)
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  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)



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Marfan Syndrome: A Primer for Clinicians and Scientists (Medical Intelligence Unit)