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Improvement of cardiac function in thalassemia major treated with L-carnitine
Amal El-Beshlawy1, Lamis Ragab, Alia Abdel Fattah
1Hematology Clinic, New Cairo University Children Hospital, Cairo, Egypt. amalelbeshlawy@yahoo.com
Insights
L-carnitine supplementation improved cardiac function in thalassemia major patients. Multigated equilibrium radionuclide angiography (MUGA) showed enhanced systolic and diastolic function after six months of treatment.
Area of Science:
- Cardiology
- Hematology
- Pharmacology
Background:
- Heart disease is a primary concern for thalassemia patients due to chronic anemia and hemosiderosis.
- Anemia and hypoxia impair myocardial energy production, affecting cardiac function.
- L-carnitine, a derivative of butyric acid, is explored for its potential to mitigate these cardiac defects.
Purpose of the Study:
- To evaluate the therapeutic effect of L-carnitine on cardiac function in patients with thalassemia major.
- To assess changes in both systolic and diastolic function following L-carnitine administration.
Main Methods:
- Cardiac function was assessed in 30 thalassemia major patients before and after 6 months of oral L-carnitine (50 mg/kg/day).
- Evaluations included echocardiography, Doppler, and multigated equilibrium radionuclide angiography (MUGA).
- Systolic and diastolic parameters were analyzed to determine treatment efficacy.
Main Results:
- Echocardiography showed no significant changes in systolic or diastolic function (p > 0.05).
- MUGA revealed significant improvements in diastolic function, including increased peak filling rate (p < 0.03) and decreased time to peak filling (p < 0.02).
- Left ventricular ejection fraction significantly increased (p = 0.0001), indicating improved systolic function.
Conclusions:
- L-carnitine demonstrates potential as an effective treatment for improving cardiac status in thalassemic patients.
- Multigated equilibrium radionuclide angiography (MUGA) proved to be the most sensitive method for assessing left ventricular function in this cohort.
Introduction:
Heart disease secondary to chronic anemia and hemosiderosis remains the major cause of morbidity and mortality in thalassemic patients. Chronic anemia and the tissue hypoxia it induces impair free fatty acid oxidation and ATP production in myocardial cells. The use of L-carnitine, a butyric acid derivative, may help overcome some of these defects.
Objective:
To investigate the effect of L-carnitine therapy on cardiac function in thalassemia major patients.
Materials And Methods:
Cardiac function was evaluated in 30 patients attending our clinic. The mean (+/-SD) age was 15.87 +/- 3.19 years. The studies we performed included echocardiography, Doppler and multigated equilibrium radionuclide angiography (MUGA). Systolic and diastolic function was evaluated before starting L-carnitine treatment and after 6 months of oral L-carnitine (50 mg/kg/day).
Results:
Echocardiography studies revealed no significant changes in systolic and diastolic function after L-carnitine therapy (p > 0.05). Analysis of the data taken by MUGA performed in 20 of the patients, however, showed a significant improvement of diastolic function after 6 months of L-carnitine therapy. The mean peak filling rate (end-diastolic volume/s) increased from 3.15 +/- 1.06 to 3.61 +/- 1.68 (p < 0.03). The time to peak (during filling) decreased significantly from 143.45 +/- 42.04 to 117.70 +/- 24.40 s (p < 0.02). Systolic function showed a significant increase in the left ventricular ejection fraction from 58.25 +/- 9.92 to 63.95 +/- 10.11% (p = 0.0001).
Conclusion:
L-carnitine may be an effective drug for improving the cardiac status of thalassemic patients. MUGA is the most accurate technique of those used here for assessing left ventricular function in these patients.
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