Review
Endovascular Treatment for Type B Dissection in Marfan Syndrome: Is It Worthwhile?

https://doi.org/10.1016/j.athoracsur.2012.09.059Get rights and content

Marfan syndrome is the most frequently inherited disorder of connective tissue and is strongly associated with aortic dilatation, dissection, and rupture; in these patients, type B dissection occurs substantially. It is not known whether stent grafting, which is now frequently used in type B aortic dissection and descending thoracic aneurysms in non-Marfan patients, is a valuable option in Marfan patients, and reports from the literature are sparse and sporadic. We performed a systematic review of studies reporting the early and late results of endovascular stent grafting in Marfan patients with type B dissection in the attempt to quantify possible benefits or potential drawbacks of this approach in these usually very sick patients. Although associated with a low operative risk (1.9%), endovascular stent grafting in patients with Marfan syndrome carries a substantial risk of early and late complications, mainly endoleaks and surgical conversions, and of death at midterm follow-up. Because these complications are relatively more frequent in patients undergoing endovascular stent grafting for chronic dissections, these data suggest caution against the routine use of endovascular stent grafting in Marfan patients.

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Search Strategy

A systematic search was performed using the PubMed database to identify all studies reporting the results and evaluating the outcome of endovascular treatment of descending acute and chronic thoracic aortic dissections in patients with Marfan disease. Original articles, case series, and individual reports published in English from January 2000 to November 2011 were considered, and we looked for all the studies reporting the outcome of patients who underwent endografting for descending thoracic

Endovascular Stent Grafting in MFS Due To Aortic Dissections

We ultimately identified 12 articles with 54 patients. One article was discarded from further analysis because of possible double publication [8]; also, because there was possible overlapping in the reports of some patients between two articles [4, 10], data for 4 patients were removed from one of the two publications [4] (data were available from the authors' institution). Overall, 40 of these patients were identified from articles focused on MFS, whereas data concerning 14 additional patients

Comment

The treatment of thoracic aortic disease has changed radically with the advances made in endovascular therapy since the concept of TEVAR was first described 15 years ago. Many studies have demonstrated excellent outcomes of TEVAR for the treatment of thoracoabdominal aortic aneurysm, with reduced reported perioperative morbidity and death compared with conventional open repair. More recently, similar outcomes have been demonstrated for the treatment of type B dissections, although some recent

References (23)

Cited by (63)

  • The Society of Thoracic Surgeons/American Association for Thoracic Surgery Clinical Practice Guidelines on the Management of Type B Aortic Dissection

    2022, Annals of Thoracic Surgery
    Citation Excerpt :

    Data regarding the use of TEVAR to treat connective tissue disorder patients with TBAD are limited to data registries and small series. Pacini and colleagues139 performed a comprehensive review of 5572 studies to assess the early and late results of TEVAR in Marfan patients with acute and chronic TBAD, revealing 12 citations with data on 54 patients (11 acute and 43 chronic). The procedural incidence of mortality, stroke, and paralysis were all 1.9%.

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These authors contributed equally to this paper.

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