Abstracts S211 Eur J Echocardiography Abstracts Supplement, December 2006 p<0.05) respect to control group; a significant higher myocardial IBS at septum (53.3±20.7 vs 43.4±7.1%, p<0.05), but comparable at the posterior wall level (33.6±13 vs 32.5±9.3%, p=ns) respect to the control group; a lower CVI in DM1 group both at septum (11.3±17.7 vs 37±9.9%, p<0.0001) and posterior wall level (23.2±17.9 vs 43.6±12.7%, p<0.0001). Conclusions: The present study demonstrated that integrated backscatter

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... lysis could be useful to detect subclinical ultrastructural and systolic functional myocardial alterations in DM1. Background: Wilson's disease is an inherited autosomal recessive disorder of copper metabolism resulting in pathological accumulation of copper in the liver, brain and other tissues. One of the reported manifestations is cardiac involvement. Purpose: The aim of the study was to evaluate structural and functional echocardiographic abnormalities in patients with Wilson's disease. Furthermore we assessed left ventricular (LV) contractility function using both traditional parameters and midwall fractional shortening (MFS). Methods: We studied 42 patients with Wilson's disease (19 men and 23 women, mean age 34±10 years) and 42 age and sex-matched healthy volunteers. All subjects underwent complete echocardiographic examination. We included only subjects with good quality echocardiograms allowing M-mode measurements. We assessed LV wall thickness, LV mass index (LVMI), relative wall thickness (RWT), ejection fraction (EF), endocardial fractional shortening (FS), MFS and MFS adjusted by circumferential end-systolic wall stress (ESS). Results: In comparison to healthy subjects patients with Wilson's disease had increased thickness of the interventricular septum (9.5±1.4 vs 8.6±1.1 mm, p<0.01) and LV posterior wall (9.1±1.3 vs 8.2±1.0 mm, p<0.01). While the two groups did not differ in LVMI, RWT was significantly increased in Wilson's disease patients compared to control subjects (0.39±0.06 vs 0.34±0.04, p<0.001). Concentric LV remodelling was present in nine patients (21%) and LV hypertrophy in one patient. Ejection fraction and FS showed a non-significant trend toward lower values (EF 62±5% vs 64±5%, p=0.06; FS 0.35±0.06 vs 0.37±0.05, p=0.06.) while MFS was significantly decreased in Wilson's disease patients compared to control subjects (0.17±0.03 vs 0.19±0.02, p=0.001). When corrected by ESS decreased MFS compared to predicted was found in 54% (n=22) of Wilson's disease patients and in 17% (n=7) of control subjects (p<0.001). The established echocardiographic abnormalities did not correlate with the type of Wilson's disease manifestation, presence of His1069Gln mutation, laboratory parameters or duration and type of therapy. Conclusions: Cardiac involvement in Wilson's disease patients is characterised by LV parietal thickening, increased prevalence of concentric LV remodelling and decreased myocardial contractility measured by MFS. Assessment of MFS may reveal significant contractility impairment masked by abnormal LV geometrical remodelling.