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Background: Systolic and diastolic dysfunction of the left ventricle (LV) are present in patients with cardiac involvement in Fabry disease. The aim of this study was to investigate the diagnostic value of the Tei-index, a marker for combined diastolic and systolic function, in patients with Fabry disease.Methods: A total of 66 genetically confirmed consecutive Fabry patients were included in this study. Standard echocardiography including Tei-index and magnetic resonance imaging (MRI) were performed. Patients were followed for 2.9±1.9 years, 56 patients receiving enzyme replacement therapy (ERT) and 10 patients had natural history follow up. Patients were subdivided in three groups: 1) patients without cardiac involvement 2) patients with LV hypertrophy and without late enhancement on MRI 3) patients with late enhancement on MRI.Results: The Tei-index was significantly higher in the groups 2 (0.56±0.10) and 3 (0.60±0.16) compared to patients without cardiac involvement (0.44±0.10; p<0.001). All patients with a Tei-Index higher than 0.64 showed signs of a cardiomyopathy. In contrast, ejection fraction was normal in all three patient groups and therefore not useful for the detection of cardiac involvement. A significant positive correlation was observed between LV wall thickness and the Tei-index in the complete patient cohort. Moreover ROC analysis revealed a large area under the curve for Tei-index and hypertrophy, while the area under the curve for fibrosis was small. Tei-index remained unchanged in the natural history and ERT group during follow up.Conclusion: In our cohort the Tei-index was of limited value to detect myocardial fibrosis and monitor ERT. However the cardiomyopathy progression towards LV hypertrophy seems to be paralleled by global functional impairment which can be assessed by the Tei-index but not by ejection fraction. Thus, the Tei-index seems to be a global parameter which can detect LV functional reduction in Fabry disease.