AORTIC BIOMECHANICAL PROPERTIES IN PEDIATRIC-PATIENTS WITH THE MARFAN-SYNDROME, AND THE EFFECTS OF ATENOLOL

The Marfan syndrome is a heritable disorder involving biochemical abnormalities of the extracellular matrix. These abnormalities underlie the absence of structural integrity of the cardiovascular system.1-4 Recently, these connective tissue abnormalities were shown to be associated with hemodynamic and biomechanical alterations of the aorta.5,6 Adults with the Marfan syndrome demonstrate abnormal elastic properties manifest by decreased aortic distensibility and an increased aortic stiffness index.6 The present investigation sought to determine both the aortic biomechanical properties of pediatric patients with the Marfan syndrome and if therapy with a β-adrenergic blocking agent had any effect on these properties. © 1993.