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on February 9, 2006

Arteriosclerosis, Thrombosis, and Vascular Biology. 2006
Published online before print February 9, 2006, doi: 10.1161/01.ATV.0000209649.60409.38
A more recent version of this article appeared on April 1, 2006
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Submitted on July 8, 2005
Accepted on January 23, 2006

Cardiac and Vascular Hypertrophy in Fabry Disease. Evidence for a New Mechanism Independent of Blood Pressure and Glycosphingolipid Deposition

Frédéric Barbey ; Noureddine Brakch ; Ales Linhart ; Nathalie Rosenblatt-Velin ; Xavier Jeanrenaud ; Salah Qanadli ; Beat Steinmann ; Michel Burnier ; Tomas Palecek ; Jan Bultas ; and Daniel Hayoz *

From the Nephrology Department (F.B., M.B.), University Hospital, Lausanne, Switzerland; Department of Vascular Medicine (M.B., N.R.-V., D.H.), University Hospital, Lausanne, Switzerland; Second Department of Internal Medicine (T.P., J.B.), 1st School of Medicine, Charles University, Prague, Czech Republic; Radiology Department (S.Q.), University Hospital, Lausanne, Switzerland; Cardiology Department (X.J.), University Hospital, Lausanne, Switzerland; Department of Metabolism and Molecular Pediatrics (B.S.), Zürich, Switzerland.

* To whom correspondence should be addressed. E-mail: daniel.hayoz{at}chuv.ch.

Objectives--Fabry disease is an X-linked disorder resulting from {alpha}-galactosidase A deficiency. The cardiovascular findings include left ventricular hypertrophy (LVH) and increased intima-media thickness of the common carotid artery (CCA IMT). The current study examined the possible correlation between these parameters. To corroborate these clinical findings in vitro, plasma from Fabry patients was tested for possible proliferative effect on rat vascular smooth muscle cells (vascular smooth muscle cell [VSMC]) and mouse neonatal cardiomyocytes.

Methods and Results--Thirty male and 38 female patients were enrolled. LVH was found in 60% of men and 39% of women. Increased CCA IMT was equally present in males and females. There was a strong positive correlation between LV mass and CCA IMT (r2=0.27; P<0.0001). VSMC and neonatal cardiomyocyte proliferative response in vitro correlated with CCA IMT (r2=0.39; P<0.0004) and LV mass index (r2=0.19; P=0.028), respectively.

Conclusions--LVH and CCA IMT occur concomitantly in Fabry suggesting common pathogenesis. The underlying cause may be a circulating growth-promoting factor whose presence has been confirmed in vitro.
Key words: Fabry disease • growth-promoting factor • intima-media thickness • left ventricular hypertrophy




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