Management of aortic valve incompetence in patients with Marfan syndrome

Abstract

Follwoing the original description of the skeletal manifestation of Marfan syndrome (1, 2), a relatively long time elapsed before recognition of the potentially lethal cardiovascular manifestations of the disease (3–5), and only recently has it become evident that these are the main determinants of prognosis of affected individuals (6). If uncorrected, these manifestations can lead to severe disability or death at a young age. Although prolapse of the mitral valve is the most common cardiovascular manifestation of the condition, pathology of the ascending aorta and root is frequently seen and is the most common manifestation requiring surgical intervention. Aortic regurgitation may result from aneurysmal dilitation or dissection. The purpose of this chapter is to describe the management of aortic regurgitation in Marfan syndrome with particular reference to the use of a valve-conserving operation combined with radical excision of the sinuses, a technique introduced by us 15 years ago, and the place of prophylactic surgical treatment which, in our view, is closely linked to valve repair.