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Biventricular function in Friedreich's ataxia: a radionuclide angiographic study
Insights
Friedreich
Area of Science:
- Cardiology
- Neurology
Background:
- Friedreich's ataxia (FA) is a rare inherited neurodegenerative disorder.
- Cardiac involvement, particularly hypertrophic cardiomyopathy, is common in FA patients.
Purpose of the Study:
- To assess systolic and diastolic ventricular function in patients with Friedreich's ataxia and hypertrophic cardiomyopathy.
- To identify specific cardiac alterations in FA patients using radionuclide angiography.
Main Methods:
- Radionuclide angiography was performed on 21 patients diagnosed with Friedreich's ataxia and hypertrophic cardiomyopathy.
- Systolic and diastolic indices of both left and right ventricles were analyzed.
Main Results:
- Most systolic function indices and right ventricular diastolic function were normal.
- Patients with FA showed a significantly prolonged time to peak filling rate relative to diastolic time in the left ventricle, especially at higher heart rates (r = 0.79).
- 48% of FA patients exhibited hypokinetic segments in the right ventricle, while left ventricular movement was minimally impaired.
Conclusions:
- Friedreich's ataxia patients exhibit altered left ventricular diastolic filling patterns, more pronounced with increased heart rate.
- Right ventricular hypokinesis is a notable finding in nearly half of FA patients and warrants monitoring due to the risk of congestive heart failure.
Abstract:
The systolic and diastolic function of both ventricles was assessed by radionuclide angiography in 21 patients with Friedreich's ataxia and hypertrophic cardiomyopathy. The indices of systolic function of the two ventricles and those of diastolic function of the right ventricle were generally normal. But in patients with Friedreich's ataxia the time to peak filling rate divided by the diastolic time of the left ventricle was significantly larger than normal. The increase correlated with the heart rate (r = 0.79) and this suggests an alteration in the timing of ventricle filling that is more evident at high heart rates. Movement of the left ventricle was little impaired; however, in 48% of the patients with Friedreich's ataxia the right ventricle showed evidence of hypokinetic segments. Because there is a tendency for congestive heart failure to develop in patients with Friedreich's ataxia, this hypokinesis of the right ventricle should be monitored at follow up.