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The cardiomyopathy in Friedreich's ataxia — New biomarker for staging cardiac involvement  Frank Weidemann, Dan Liu, Kai Hu, Cristiane Florescu, Markus.

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Presentation on theme: "The cardiomyopathy in Friedreich's ataxia — New biomarker for staging cardiac involvement  Frank Weidemann, Dan Liu, Kai Hu, Cristiane Florescu, Markus."— Presentation transcript:

1 The cardiomyopathy in Friedreich's ataxia — New biomarker for staging cardiac involvement 
Frank Weidemann, Dan Liu, Kai Hu, Cristiane Florescu, Markus Niemann, Sebastian Herrmann, Bastian Kramer, Stephan Klebe, Kathrin Doppler, Nurcan Üçeyler, Christian Oliver Ritter, Georg Ertl, Stefan Störk  International Journal of Cardiology  Volume 194, Pages (September 2015) DOI: /j.ijcard Copyright © Terms and Conditions

2 Fig. 1 Overview of the key cardiac parameters characterizing the cardiomyopathy (CM) stages. The evaluated parameters were a) T-wave inversion in resting electrocardiography (yes/no) for depolarization abnormality, b) increased end-diastolic posterior wall thickness (LVPWT, ≥11mm) for hypertrophy, c) late enhancement positive myocardium (yes/no) for replacement fibrosis, d) elevated high sensitive troponin (hsTNT, ≥14ng/ml) for myocyte damage, and e) decreased ejection fraction (EF, <55%) for global LV function. International Journal of Cardiology  , 50-57DOI: ( /j.ijcard ) Copyright © Terms and Conditions

3 Fig. 2 Individual data for the change of averaged left ventricular end-diastolic wall thickness (septum+posterior wall) during 5years of follow-up. Slopes are shown in relation to the cardiomyopathy (CM) stages. International Journal of Cardiology  , 50-57DOI: ( /j.ijcard ) Copyright © Terms and Conditions

4 Fig. 3 A typical example of a 26year-old female Friedreich's ataxia patient with severe cardiomyopathy. Left upper: T-wave inversion observed on resting electrocardiography. Right upper: Patchy late enhancement positive regions in the left ventricle (LV) assessed by cardiac magnetic resonance imaging. Left lower: Retrospective M-mode echocardiography showing LV hypertrophy with an end-diastolic wall thickness of 13mm (ejection fraction=66%). Right lower: M-mode echocardiography after 5years of follow-up shows a decrease of end-diastolic wall thickness to 10mm (ejection fraction=60%). In addition, hsTNT was elevated (16.5ng/ml) in this patient. International Journal of Cardiology  , 50-57DOI: ( /j.ijcard ) Copyright © Terms and Conditions


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