[Cardiovascular pathology in Marfan syndrome. Study of 11 children using two-dimensional echocardiography]

From 1983 to 1987 we have studied by two-dimensional echocardiography 11 pediatric patients with Marfan's syndrome. All of them presented cardiovascular lesions localized mainly in the aortic and mitral valves and in the ascending aorta. Nine patients had a dilated aortic ring in both transversal and longitudinal diameters 2.03 and 2.29 cm/m2 of body surface (bs), respectively. The average value of the aortic valvular area was 4.71 cm2/m2 bs. The 9 patients presented also dilatation of the ascending aorta (2.01 cm/cm2 bs). There was thickening of the mitral valve in 5 cases and a prolapse was present in 8 (73%). In 4 cases the prolapse was localized in anterior leaflet valve in 2 in the posterior and in 2 in both. The maximal mitral valve diastolic area was 3.25 cm2/m2 bs. None of them has needed during follow up valvular replacement. Considering the importance of this cardiovascular disease and knowing its unpredictable evolution we recommend, in the absence of valvular insufficiency, and annual echocardiographic reevaluation, and more often if valvular insufficiency is already present.

two-dimensional echocardiographic is useful and necessary for the diagnosis and follow up of patients with Marfan's syndrome.