Summary

Marfan’s syndrome is a heritable multisystem disorder with a highly variable clinical presentation.

This study was designed to characterize the spectrum of clinical, anatomical and treatment profiles in patients with Marfan’s syndrome.

A serie of 49 patients, 36 male, aged 7-71years has been evaluated. Thirty three percent had manifestations in ocular system, 41% had skeletal manifestations and all patients had cardiac disease. Thirty seven patients underwent cardiac surgery: in twenty-nine patients with aortic disease Bentall de Bono technique was used, three descending aortic surgery, two aortic valve replacement, one mitral valve replacement, one atrial septal defect repair and one aortic coarctation repair. There were 8 postoperative deaths and 2 in the clinical group.

In patients with Marfan’s syndrome with a high rate of cardiac complications, serial noninvasive imaging should be encouraged. Early diagnosis and rigorous follow-up can help prevent cardiac complications in this population.