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High-Dose Beta-Blocker Hypertrophic Cardiomyopathy Therapy in a Patient with Friedreich Ataxia

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Abstract

Heart involvement in patients with Friedreich ataxia (FRDA) is a condition marked by inevitable progressive deterioration, with premature death. There is currently no specific treatment for FRDA. Recently, a therapy with idebenone, a short-chain coenzyme Q10 analogue, was reported to reduce left ventricular mass by 20% in about half of FRDA patients, but a double-blind placebo-controlled study did not fully confirm these results. In this case report, we present a 5-year follow-up of symmetrical concentric hypertrophic cardiomyopathy (HCM) in an FRDA patient treated with high-dose propranolol. The therapy resulted in a reduction in the thickness of the septal and posterior left ventricular walls and complete normalization of diffuse electrocardiographic repolarization abnormalities. To the best of our knowledge, this is the first such case to be reported in the literature, demonstrating the positive effects of high-dose beta-blocker treatment on heart involvement in patients with FRDA.

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Article Open access 10 May 2019

Sven Dittrich, Erika Graf, … on behalf of the German Competence Network for Congenital Heart Defects and the Treat-NMD Neuromuscular Network Investigators list of additional local Investigators and co-workers of the German Competence Network for Congenital Heart Defects and the Treat-NMD Neuromuscular Network

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Authors and Affiliations

  1. Department of Pediatric Cardiology, Mother and Child Health Institute, 6–8 R. Dakica Street, 11070, Belgrade, Serbia and Montenegro

    J. Kosutic

  2. Department of Pediatric Neurology, Mother and Child Health Institute, 6–8 R. Dakica Street, 11070, Belgrade, Serbia and Montenegro

    D. Zamurovic

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  1. J. Kosutic
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  2. D. Zamurovic
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Correspondence to J. Kosutic.

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Kosutic, J., Zamurovic, D. High-Dose Beta-Blocker Hypertrophic Cardiomyopathy Therapy in a Patient with Friedreich Ataxia. Pediatr Cardiol 26, 727–730 (2005). https://doi.org/10.1007/s00246-005-0930-7

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  • DOI: https://doi.org/10.1007/s00246-005-0930-7

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