Abstract

Marfan syndrome (MFS) is the most common inherited connective tissue disease and is characterised by mutations of the fibrillin 1 (FBN1) gene encoding the protein fibrillin-1 [1]. MFS carries an increased risk of aortic dilatation, dissection and rupture, which are responsible for increased and early mortality rate.Prevention of aortic dissection is one of the cornerstones of care in patients with Marfan syndrome and prophylactic surgical replacement of dilated aortic root and/or ascending aorta has significantly increased life expectancy of patients with Marfan syndrome. In a worst case scenario of aortic dissection, open surgery is the treatment of choice in the setting of MFS regardless of type and location [2].