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(Circulation. 1995;91:1272-1274.)
© 1995 American Heart Association, Inc.

Articles

Ultrasonic Characterization of the Aortic Architecture in Marfan Patients

Dianna M. Milewicz, MD, PhD

From the Department of Internal Medicine, University of Texas–Houston Medical School, Houston, Tex.

Correspondence to Dianna M. Milewicz, MD, PhD, University of Texas–Houston Medical School, MSB 1.614, 6431 Fannin, Houston, TX, 77030.

Key Words: Editorials • ultrasonics • aorta

	Introduction

 
Components found within the media of the aortic wall allow the aorta to withstand the mechanical stresses it is subjected to throughout life. Elastic fibers and collagen found in the aortic media form the stress-distributing and stress-bearing systems, respectively, whereas smooth muscle cells provide an active contractile component. These structural components are arranged in a highly ordered manner within the media, with circumferential smooth muscle cell layers alternating with interposing elastic laminae.^{1 2 3}

Elastic fibers consist of two morphologically distinct components: an amorphous core of elastin and a peripheral mantle of microfibrils.⁴ Elastin is the predominant protein component of the core of the elastic fiber and endows the fiber with the characteristic property of elastic recoil. Microfibrils are fine extracellular filaments that are visible only by electron microscopy. The microfibrils in the elastic fiber are typically 10 to 12 nm in diameter and are composed primarily of fibrillin proteins. These proteins are coded for by two closely related genes, FBN1 and FBN2, and are characterized by a repeated domain structure.^{5 6 7} The molecular interactions involved in the polymerization of fibrillin to form the microscopically visible microfibrils remain to be defined. All the proteins involved in the formation of microfibrils have not yet been identified, nor is it known how these proteins interact at the molecular level to form microfibrils. It appears that fibrillin-containing microfibrils are crucial for proper elastic fiber system development. Studies of the developing aorta show that microfibrils appear initially in the aortic media, followed by the deposition of elastin . . . [Full Text of this Article]