title: Arterial hemodynamic indexes in Marfan's syndrome.
abstract: Aortic impedance, wave reflection magnitude, and compliance were measured in patients with Marfan's syndrome during diagnostic cardiac catheterization. Impedance and wave reflections were calculated from standard Fourier series analysis of ascending aorta micromanometer pressure and electromagnetic flow records. Compliance was estimated by a method recently proposed that uses the area under the pressure-time curve assuming a two-element Windkessel model of the circulation. Measurements were made in the baseline state, during vasodilatation with nitroprusside, after beta-adrenergic receptor blockade with intravenous propranolol, and during vasodilatation after beta-blockade. Marfan's syndrome produces alterations from normal in some hemodynamic variables: during baseline conditions, the magnitude of wave reflection was higher than in normal patients. This was normalized by vasodilatation and further increased by beta-blockade. Despite the greatly dilated aortic root, the aortic characteristic impedance was in the normal range, suggesting increased aortic wall stiffness. The baseline total arterial compliance was greatly increased with nitroprusside and was reduced by beta-blockade. beta-Blockade did not decrease the maximum acceleration of blood into the ascending aorta. These are the first detailed measurements of hemodynamic indexes in this disease. The results suggest that acute beta-blockade is not necessarily beneficial in reducing hemodynamic loading on an already dilated aortic root.
authors.affiliation: Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: F C
authors.initials: FC
authors.lastname: Yin
authors.forename: K P
authors.initials: KP
authors.lastname: Brin
authors.forename: C T
authors.initials: CT
authors.lastname: Ting
authors.forename: R E
authors.initials: RE
authors.lastname: Pyeritz
mesh_terms: D000328:Adult; D001011:Aorta / Q000503:physiopathology*; D001783:Blood Flow Velocity; D006328:Cardiac Catheterization; D005260:Female; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000503:physiopathology*; D009599:Nitroprusside; D011433:Propranolol; D011673:Pulsatile Flow; D014655:Vascular Resistance
chemical_list: D009599:Nitroprusside; D011433:Propranolol