To present the outcome of thoracic endovascular aortic repair (TEVAR) for the treatment of aortic dissection in patients with Marfan syndrome.

From March 2003 to December 2008, TEVAR with percutaneous stent-graft placement was performed in 5 patients (2 men and 3 women; mean age 44.8 years, range 35-58) for the treatment of aortic dissection in patients with Marfan syndrome. Four patients undergone previous previous surgical aortic root repair due to type I aortic dissection (type III at the time of TEVAR), and one had type III without surgical history.

Clinical failure was occurred in two of five patients. One failure of TEVAR was due to immediate complication of a new retrograde dissection from inadvertent guide wire working during the procedure. Surgical conversion was done successfully. The other failure was a case of delayed complication of pseudoaneurysm at the proximal end of the stent-graft. In the remaining three patients, primary entry tear was excluded completely with false lumen thrombosis without any problem.

Although there is a controversy of the role of TEVAR in patients with Marfan syndrome, TEVAR is feasible in selected patients. However, particular attention in terms of case selection, design of stent-graft, vigilant procedure as well as meticulous follow-up should be considered.

Marfan syndrome, Aortic dissection, Stent-graft

A. Progressive enlargement of the descending thoracic aorta due to type III aortic dissection. B. During the procedure, the guide wire was negotiated inadvertently into the aortic wall (arrows). C. Post-placement of aortic stent-graft aortography shows a new intimal flap (arrows) created by inadvertent guide wire. D. Post-procedure CT scan demonstrates new retrograde dissection and persistent false lumen flow. The patient was converted to surgery.

The patient underwent Bentall operation due to type I aortic dissection. And then, type III aortic dissection was treated with percutaneous stent-graft. However, a new pseudoaneurysm (A, arrows) at the proximal end (B, arrow) of the stent was developed three and a half year later the stent-graft procedure.

The patient had a history of Bentall operation due to type I aortic dissection. A. Initial CT scan shows type I aortic dissection and annuloaortic ectasia. B, C. Aortography after Bentall operation shows type III aortic dissection. D, E, F. CT scan before stent-graft shows type III aortic dissection. G, H, I. Follow-up CT scan 6 years after the stent-graft shows complete exclusion of the entry tear and complete thrombosis at proximal descending thoracic aorta without any complication.

The Marfan syndrome is a heritable disorder of the connective tissue which affects the cardiovascular, ocular, and skeletal system. The cardiovascular manifestation with aortic root dilatation, aortic valve regurgitation, and aortic dissection has a prevalence of 60% to 90% and determines the premature death of these patients. Thirty-four percent of the patients with Marfan syndrome will have serious cardiovascular complications requiring the treatment in the first 10 years after diagnosis. Before aortic surgery became available, the majority of the patients died by the age of 32 years. Introduction in the aortic surgery techniques caused an increase of the 10 year survival rate up to 97%. There are several reports of TEVAR for the treatment of aortic dissection in patients with Marfan syndrome. However, the reports are small number of case series, the long-term results are not available yet, and complications such as a new dissection and pseudoaneurysm related with the stent were reported which make it is hard to justify the role of TEVAR in Marfan patients. The fragility of the aortic wall and disease progression may predispose to these complications. In summary, TEVAR in patients with Marfan syndrome (connective tissue disease) is feasible but still questionable regarding their young age and the rates of complications, endoleaks and reintervention due to disease progression. To prevent these complications, particular attention in terms of case selection, design of stent-graft, vigilant procedure as well as meticulous follow-up should be considered.

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