Cardiovascular Involvement in Marfan Syndrome
Mitral valve problems and aortic root dilatation are nearly universal in patients with Marfan syndrome. Using echocardiography, Pan et al demonstrated mitral prolapse in 12 of 12 and aortic root dilatation in 10 of 12 patients with Marfan syndrome. These lesions are also present in over one fourth of the patients' first degree relatives. Geva et al found echocardiographic features of mitral prolapse in 25 of 25 patients with Manfan syndrome, and aortic root dilatation in 20(80%).
The studies by Geva et al and by Pyertiz and Wappel demonstrate that mitral valve dysfunction and aortic root dilatation are progressive. Morbidity and mortality are more likely to be due to mitral insufficiency in young subjects and to aortic dissection or aortic insufficiency in older patients.
