The Marfan syndrome is an inherited disorder of the connective tissue which is 
mainly caused by a mutation in the fibrillin-1 gene. The defect in the 
connective tissue protein can lead to several organ dysfunctions. For the life 
expectancy, the cardiovascular aspect is of paramount importance. Patients with 
Marfan syndrome may develop aortic aneurysms and valvular heart defects. The 
risk of aortic aneurysms consists in the development of aortic dissection or 
rupture with their fatal consequences. Besides the cardiovascular manifestation, 
the skeletal and ocular system can also be affected. The skeletal manifestation 
is often characterised by long limbs, arachnodactyly, and abnormal joint 
flexibility along with other signs. Patients may also have dislocated lenses, 
ectasia of the dural sac, stretch marks, spontaneous pneumothorax, recurrent 
hernia, or a family history suspicious for Marfan. During the past years, other 
related connective tissue disorders with analogous organ manifestation have been 
described (e.g., Loeys-Dietz syndrome). In this article we present the basic 
knowledge about these connective tissue disorders, and we mention new insights 
in the recently explored pathophysiology of the disorder which is a possible 
target for future medical treatment options. Furthermore, recent new concepts 
for the prophylactic treatment of the aortic manifestation are explained.