Left Ventricular Diastolic Dysfunction in Children and Young Adults with Marfan Syndrome

摘要

Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.