P1532 Segmental assessment of left ventricle in patients with Fabry"s disease

Anderson-Fabry disease (AFD) is a rare X-linked metabolic disorder due to deficiency in lysosomal enzyme activity of a-galactosidase A, resulting in pathological accumulation of glycosphingolipids in several tissues and a progressive multi-organ dysfunction. The Global Longitudinal Strain (GLS) of the left ventricle (LV) by Speckle-tracking echocardiography (STE) has been show to detect subclinical cardiac involvement in many cardiomyopathies. The Mechanical Dispersion (MD), derived from STE is considered able to reflect a heterogeneous myocardial contraction, evaluated in many cardiopathies. A reduction in GLS was associated with myocardial fibrosis in the subclinical stages of AFD cardiomyopathy, but there is no MD data in AFD. The aim of the study was to evaluate the distribution of GLS and MD of LV in patients with AFD. 47 consecutive AFD adult patients treated (37F, 12 M, age 45 + 17 years) were examined by a complete echocardiographic examination from a Vivid E95 ultrasound scanner (Horten, Norway). The STE post-processing was performed by Echopac 2.02, in apical long-axis, 4-chambers, and 2-chambers views to determine GLS and MD. Data were expressed as mean± standard deviation. The comparison between the AFD group and 20 normal subjects (N) was performed with unpaired T-test. Compared to N, the AFD group showed higher values of LVMi, LAVi, E/E’, and MD, but a lower value of GLS (Table). GLS was significantly lower in the basal (sept p<.002; post p<.0001) and mid segments (ant-sept p<.008; post p<.0001; ant p<.001). MD was significantly higher in the inferior (basal p<.003; mid p<.01; apical p<.005) and lateral segments (mid p<.004; apical p<.001). In patients with AFD, MD added to GLS seems to be a promising tool for the early diagnosis of segmental dysfunction . Table1: Results AFD N p< m SD m SD LVMi g/sqm 77.1 29.8 62.3 14.1 0.008 LVEF % 65.7 5 63.6 3.5 0.04 LAVi ml/sqm 25.5 11 18.2 4.5 0.000 E/E" 8.3 3 5.4 1.1 0.000 TV vmax m/s 2.2 0.4 2.1 0.3 - LV GLS % -17 4 -20 2 0.003 LV MD ms 52 38 29 8 0.000

Neurologic Thoracic Outlet Syndrome: A Case Report and Clinical Review

An avid weightlifter presented with complaints of right arm and hand numbness. His workup was consistent with neurologic thoracic outlet syndrome with severe conduction block at the level of the supraclavicular fossa. He chose conservative treatment, which focused on correction of thoracic and cervical segmental dysfunction, upper crossed syndrome muscle imbalances, and upper trunk and anterior forward head postural concerns. Upon completion of the therapy program and continuation of a home exercise program, his conduction block and symptoms resolved. Conservative treatment may be an effective alternative to surgery for neurologic thoracic outlet syndrome.

Nightmare-Induced Atypical Midventricular Tako-Tsubo Cardiomyopathy

Tako-Tsubo cardiomyopathy (TTC) is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare) in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis.

The Preoperative Spine: Diagnostic Work up with fMRI

The advantages of the upright, open, multi-positional MRI, i.e. functional MRI (fMRI) are presented with illustrative cases and the literature is reviewed. In cases of not explained and especially in position-dependent spinal and/or neurological pain and/or dysfunction functional or dynamic MRI is able to reveal dynamic compressions/stenosis, as well as segmental dysfunction and instabilities in a noninvasive way. This new technology could become mandatory for the preoperative investigation of unexplained, mainly “dynamic, mobile or unstable” spinal pathologies. Allowing a better correlation of signs and symptoms with the imaging findings and thus a more targeted treatment, fMRI could help to decrease the rate of failed back surgery syndrome in the near future.