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Article

Significance of NT-proBNP and High-Sensitivity Troponin in Friedreich Ataxia

1
Cardiology Department, AP-HP, Sorbonne Université, Pitié-Salpêtrière University Hospital, 75013 Paris, France
2
ICAN (Institute for Cardiometabolism and Nutrition), Pitié-Salpêtrière University Hospital, 75651 Paris, France
3
ACTION (Allies in Cardiovascular Trials Initiatives and Organized Networks) Group, URC Lariboisière University Hospital, 75475 Paris, France
4
Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Sorbonne Université, Pitié-Salpêtrière University Hospital, 75013 Paris, France
5
Metabolic Biochemistry Department, Sorbonne Université, Pitié-Salpêtrière University Hospital, 75013 Paris, France
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(6), 1630; https://doi.org/10.3390/jcm9061630
Received: 29 April 2020 / Revised: 22 May 2020 / Accepted: 25 May 2020 / Published: 28 May 2020
(This article belongs to the Special Issue Clinical and Research of Genetic Cardiomyopathies)
Background: Friedreich’s ataxia (FA) is a rare autosomal recessive mitochondrial disease resulting of a triplet repeat expansion guanine-adenine-adenine (GAA) in the frataxin (FXN) gene, exhibiting progressive cerebellar ataxia, diabetes and cardiomyopathy. We aimed to determine the relationship between cardiac biomarkers, serum N-terminal pro-brain natriuretic peptide (NT-proBNP), and serum cardiac high-sensitivity troponin (hsTnT) concentrations, and the extent of genetic abnormality and cardiac parameters. Methods: Between 2013 and 2015, 85 consecutive genetically confirmed FA adult patients were prospectively evaluated by measuring plasma hsTnT and NT-proBNP concentrations, electrocardiogram, and echocardiography. Results: The 85 FA patients (49% women) with a mean age of 39 ± 12 years, a mean disease onset of 17 ± 11 years had a mean SARA (Scale for the Assessment and Rating of Ataxia) score of 26 ± 10. The median hsTnT concentration was 10 ng/L (3 to 85 ng/L) and 34% had a significant elevated hsTnT ≥ 14 ng/L. Increased septal wall thickness was associated with increased hsTnT plasma levels (p < 0.001). The median NT-proBNP concentration was 31 ng/L (5 to 775 ng/L) and 14% had significant elevated NT-proBNP ≥ 125 ng/L. Markers of increased left ventricular filling pressure (trans mitral E/A and lateral E/E’ ratio) were associated with increased NT-proBNP plasma levels (p = 0.01 and p = 0.01). Length of GAA or the SARA score were not associated with hsTnT or NT-proBNP plasma levels. Conclusion: hsTnT was increased in 1/3 of the adult FA and associated with increased septal wall thickness. Increased NT-proBNP remained a marker of increased left ventricular filling pressure. This could be used to identify patients that should undergo a closer cardiac surveillance. View Full-Text
Keywords: Friedreich ataxia; hs troponin; NT-proBNP; cardiomyopathy Friedreich ataxia; hs troponin; NT-proBNP; cardiomyopathy
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MDPI and ACS Style

Legrand, L.; Maupain, C.; Monin, M.-L.; Ewenczyk, C.; Isnard, R.; Alkouri, R.; Durr, A.; Pousset, F. Significance of NT-proBNP and High-Sensitivity Troponin in Friedreich Ataxia. J. Clin. Med. 2020, 9, 1630. https://doi.org/10.3390/jcm9061630

AMA Style

Legrand L, Maupain C, Monin M-L, Ewenczyk C, Isnard R, Alkouri R, Durr A, Pousset F. Significance of NT-proBNP and High-Sensitivity Troponin in Friedreich Ataxia. Journal of Clinical Medicine. 2020; 9(6):1630. https://doi.org/10.3390/jcm9061630

Chicago/Turabian Style

Legrand, Lise, Carole Maupain, Marie-Lorraine Monin, Claire Ewenczyk, Richard Isnard, Rana Alkouri, Alexandra Durr, and Francoise Pousset. 2020. "Significance of NT-proBNP and High-Sensitivity Troponin in Friedreich Ataxia" Journal of Clinical Medicine 9, no. 6: 1630. https://doi.org/10.3390/jcm9061630

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