DIAGNOSTIC-IMAGING OF THE CARDIOVASCULAR-SYSTEM IN THE MARFAN-SYNDROME

The effectiveness of current medical and surgical interventions for th e cardiovascular complications of the Marfan syndrome is highly depend ent upon data derived from noninvasive diagnostic imaging techniques. Indeed, such studies are inherent to the initial diagnostic evaluation , guide preventative medical management, allow the appropriate timing of surgical intervention, and are a standard component of the follow-u p assessment. Standard transthoracic echocardiography provides an effe ctive means to identify aortic root dilatation in the Marfan syndrome. The availability of standardized techniques and body size- and age-de pendent normative values allows accurate assessment for the presence a nd progression of aortic root dilatation. While transthoracic echocard iography is also effective in the evaluation for aortic regurgitation, mitral valve prolapse, and mitral regurgitation, its use is limited f or the detection of aortic dissection. Superior methods include contra st aortography, MRI scanning, contrast CT scanning and transesophageal echocardiography. This latter technique is also particularly useful f or the intraoperative assessment of mitral valve anatomy and function. MRI and CT scanning may be useful adjuncts in the surveillance for di sease in more distal segments of the aorta.