Treating the Thoracic Aorta in Marfan Syndrome: Surgery or TEVAR?

Abstract

Marfan syndrome (MFS) is an inherited disorder of connective tissue that is historically associated with high mortality due to disorders of the cardiovascular system. Over the past 40 years, surgery to the aortic root and thoracoabdominal aorta has resulted in a significantly prolonged life expectancy. More recently, techniques for endovascular repair of the aortic complications of MFS have been described. In this article we review the relative merits of open and endovascular approaches to thoracic aortic complications in MFS, comparing outcomes from contemporary literature. A reasonably large body of evidence has developed concerning the surgical treatment of the aortic root, ascending thoracic aorta, and arch in MFS. It is clear from large series that pathology, such as acute type A dissections and aneurysmal dilatation, can be successfully repaired with low mortality. Recently published series, although reporting smaller numbers, have demonstrated that the same is true for open surgical replacement of descending thoracic and thoracoabdominal aortic aneurysms. The evidence for thoracic endovascular aortic repair in MFS is much more limited. Small series and registry or case reports describe a heterogeneous group of patients with only short-term follow-up. Outcomes of endovascular repair are mixed, with questionable longevity. Reoperation is, however, common in MFS, and minimally invasive techniques may provide a bridging role or alternative solution when revisiting the hostile surgical field.