Marfan syndrome is a dominantly inherited connective tissue disease 
characterized by cardiovascular, skeletal and ocular manifestations, which was 
firstly described by Antoine Marfan in 1896. The underlying disorder is a 
mutation, which impairs fibrillin synthesis and is associated with the FBN-1 
gene on the 15th chromosome. Ghent Nosology is used for diagnosis. The 
progressive dilatation of the proximal aorta leading to dissection and rupture 
is the typical feature of the disease. Aortic aneurysm and aortic rupture are 
the lethal complications of the disorder. Increased life expectancy depends on 
the control and the prevention of the cardiovascular complications. The most 
frequent cardiovascular manifestation is the mitral valve involvement. The 
principal pathologic findings on the mitral valve are annular dilatation, 
fibromyxomatous changes of the leaflets and chordae, elongation and rupture of 
chordae and deposition of calcium. Prolapsus occurs in 80% of the cases. 
Elective surgery with optimal timing is associated with increased survival. In 
the follow-up of patients after surgery for aortic aneurysm and dissection, 
serial imaging studies in order to detect a new onset aneurysm or dissection on 
different sites of the aorta are essential.