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Myocardial Perfusion Reserve in Children with Friedreich Ataxia
Jeffrey A Hutchens1, Tiffanie R Johnson1,2, R Mark Payne3,4,5
1Indiana University School of Medicine, Indianapolis, IN, USA.
Insights
Friedreich
Area of Science:
- Cardiology
- Neurology
- Pediatrics
Background:
- Friedreich's ataxia (FA) poses significant perioperative risks for children due to unpredictable heart failure.
- Current methods lack clarity in identifying high-risk FA patients for surgery.
Purpose of the Study:
- To investigate the utility of myocardial perfusion reserve (MPR) as a tool for assessing surgical risk in pediatric FA patients.
- To determine if MPR can detect early cardiac compromise in children with FA.
Main Methods:
- Retrospective analysis of seven pediatric FA patients (ages 8-17) undergoing regadenoson-stress MPR testing.
- Assessed endocardial perfusion, cardiac troponin I levels, ejection fraction, and insulin levels.
Main Results:
- Six of seven FA patients exhibited impaired endocardial perfusion during stress testing.
- The same six patients showed elevated cardiac troponin I, indicating myocardial damage.
- No patients had reduced ejection fraction or elevated insulin levels.
Conclusions:
- Children with FA develop MPR defects early in the disease course.
- MPR may serve as a sensitive indicator of cardiac compromise in FA.
- MPR could aid in surgical management decisions for pediatric FA patients.
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.
