Diagnostic imaging of the cardiovascular system in the Marfan syndrome
Cause of Death
Signal Processing, Computer-Assisted
The effectiveness of current medical and surgical interventions for the cardiovascular complications of the Marfan syndrome is highly dependent 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-up assessment. Standard transthoracic echocardiography provides an effective means to identify aortic root dilatation in the Marfan syndrome. The availability of standardized techniques and body size- and age-dependent normative values allows accurate assessment for the presence and progression of aortic root dilatation. While transthoracic echocardiography is also effective in the evaluation for aortic regurgitation, mitral valve prolapse, and mitral regurgitation, its use is limited for the detection of aortic dissection. Superior methods include contrast aortography, MRI scanning, contrast CT scanning and transesophageal echocardiography. This latter technique is also particularly useful for the intraoperative assessment of mitral valve anatomy and function. MRI and CT scanning may be useful adjuncts in the surveillance for disease in more distal segments of the aorta.