The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography.

Abstract

Use of echocardiography has dramatically changed the way in which patients with Marfan syndrome are diagnosed, monitored and treated. Owing to the lethal nature of aortic complications, priority has been given to the assessment of the aortic root. Echocardiographic studies on patients with Marfan syndrome have also provided data supporting primary myocardial involvement, although this evidence has remained controversial for several years. Use of more sensitive ultrasound techniques has demonstrated mild myocardial impairment in these patients. Biventricular function assessment should be added to the aortic root evaluation, so that appropriate treatment may be offered to support myocardial function.