CASE REPORTS  VASCULAR SECTION RECENT ADVANCES IN THE MANAGEMENT OF THORACIC DISSECTIONS 

The Journal of Cardiovascular Surgery 2010 October;51(5):693-9

Copyright © 2010 EDIZIONI MINERVA MEDICA

language: English

Marfan Syndrome: when to operate TAA(A)s?

Greiner A. ¹, Grommes J. ¹, Lancer S. ¹, Autschbach R. ², Jacobs M. J. ¹ ✉

¹ European Vascular Center Aachen-Maastricht, University Hospitals Aachen, Maastricht, the Netherlands 2 Department of Thoracic and Cardiovascular Surgery University Hospital Aachen, Maastricht, the Netherlands

Marfan Syndrome is a heritable disorder of connective tissue leading to aortic aneurysms and other cardiovascular complications associated with reduced life expectancy. Marfan patients with thoracic aortic aneurysms (TAAs) or with thoracoabdominal aortic aneurysms (TAAAs) should be treated by means of open surgery, requiring an extensive protocol, including extracorporeal circulation, neuromonitoring and adjunctive modalities to provide organ protection. Then, open surgical repair of TAA(A)s are associated with excellent results. However, in the last time a gradual change to endovascular treatment in Marfan patients is observable. Particularly in patients with an increased surgical risk due to redo sternotomy or thoracotomy, endovascular treatment might be an alternative due to its less invasive approach. Consequently, thoracic endovascular aortic repair comprises a therapeutic alternative in individual situations even in Marfan patients, when the landing zones are safe and appropriate. In cases of failed endovascular therapy, however, conversion to open surgery remains still an option with acceptable results, although the distal and proximal clamping positions change inappropriate with larger extensions due to the aortic stent.