Syndrome de Marfan et réanimation

Marfan syndrome is a rare genetic disease, dominant autosomal, leading to fragility of aortic wall and other tissues. The main risk is aortic dissection and this risk is proportional to aortic dilation, so that it can be prevented by regular aortic follow-up with echocardiography, beta-blockade, and prophylactic aortic surgery. Nevertheless, aortic dissection has to be looked for using echocardiography, NMR or CT scanner if a doubt exists. A pneumothorax can also be responsible for chest pain. Other emergencies are ophtalmological. Because of aortic wall fragility, blood pressure variations have to be avoided as much as possible. Modern management has led to a substantial increase in the life expectancy of these patients.