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Myocardial Perfusion Reserve in Children with Friedreich Ataxia

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Abstract

Children with Friedreich’s ataxia (FA) are at risk of perioperative morbidity and mortality from severe unpredictable heart failure. There is currently no clear way of identifying patients at highest risk. We used myocardial perfusion reserve (MPR), an MRI technique used to assess the maximal myocardial blood flow above baseline, to help determine potential surgical risk in FA subjects. In total, seven children with genetically confirmed FA, ages 8–17 years, underwent MPR stress testing using regadenoson. Six of the seven demonstrated impaired endocardial perfusion during coronary hyperemia. The same six were also found to have evidence of ongoing myocardial damage as illustrated by cardiac troponin I leak (range 0.04–0.17 ng/mL, normal < 0.03 ng/mL). None of the patients had a reduced ejection fraction (range 59–74%) or elevated insulin level (range 2.46–14.23 mCU/mL). This retrospective study shows that children with FA develop MPR defects early in the disease process. It also suggests MPR may be a sensitive tool to evaluate underlying cardiac compromise and could be of use in directing surgical management decisions in children with FA.

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Authors and Affiliations

  1. Indiana University School of Medicine, Indianapolis, IN, USA

    Jeffrey A. Hutchens, Tiffanie R. Johnson & R. Mark Payne

  2. Division of Pediatric Cardiology, Riley Hospital for Children, Indiana University School of Medicine, 1044 West Walnut St, Room R4-302b, Indianapolis, IN, 46202, USA

    Tiffanie R. Johnson & R. Mark Payne

  3. Herman B Wells Center for Pediatric Research, Indianapolis, IN, USA

    R. Mark Payne

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  1. Jeffrey A. Hutchens
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  2. Tiffanie R. Johnson
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Correspondence to R. Mark Payne.

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Hutchens, J.A., Johnson, T.R. & Payne, R.M. Myocardial Perfusion Reserve in Children with Friedreich Ataxia. Pediatr Cardiol 42, 1834–1840 (2021). https://doi.org/10.1007/s00246-021-02675-1

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