Abstract 11846: Increased Aortic Stiffness in Patients with Aneurysms-Osteoarthritis Syndrome
Introduction The aneurysms-osteoarthritis syndrome (AOS) caused by smad3 mutations is characterized by widespread aneurysms, dissections and arterial tortuosity in thorax, abdomen, neck and intracranial arteries. At a microscopic level the aortic wall of AOS patients showed fragmentation and loss of elastic fibers, mucoid medial degeneration and accumulation of collagen. We investigated whether non-invasive arterial stiffness measurements were abnormal.
Methods Aortic stiffness was assessed by carotid-femoral pulse wave velocity (PWV) (SphygmoCor® system, ArtCor, Sydney, Australia). Common carotid distensibility was measured by means high-precision echo-tracking device (Wall Track System, Pie-medical Esaote). Mean PWV values were compared with reference values from the Expert Consensus Document. Since reference values for carotid distensibility measurements are not available, we also performed these measurements in sex-, age- and smoking status matched controls.
Results Eighteen AOS patients and 18 matched controls (mean age 37 ± 13 years) were included in this study. Mean values of aortic PWV were 9.0 ± 2.3 m/s. When compared to references values, 6/18 patients (33%) had PWV values >2 standard deviations above corresponding mean values. There was no correlation between aortic PWV and aortic diameter at the level of the Valsalva sinuses (r=-0.278; p=0.357). Table 1 shows that carotid distensibility in AOS patients did not differ significantly from matched controls.
Conclusion In conclusion, aortic stiffness was increased in patients with AOS, as was previously described for other disorders affecting the aorta, such as Marfan syndrome. There was no correlation between aortic stiffness and aortic diameter, suggesting that arterial stiffness occurs irrespectively of aneurysm formation. Local measures of arterial stiffness as the carotid distensibility did not seem to be affected by this mutation.
- © 2011 by American Heart Association, Inc.