Improvement of cardiac function in thalassemia major treated with L-carnitine.
BACKGROUND
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.


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).


CONCLUSIONS
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.
El-Beshlawy
Amal
A
Hematology Clinic, New Cairo University Children Hospital, Cairo, Egypt. amalelbeshlawy@yahoo.com
Ragab
Lamis
L
Fattah
Alia Abdel
AA
Ibrahim
Ilham Youssry
IY
Hamdy
Mona
M
Makhlouf
Ali
A
Aoun
Elie
E
Hoffbrand
Victor
V
Taher
Ali
A
D000293:Adolescent; D000328:Adult; D000792:Angiography / Q000187:drug effects; D002331:Carnitine / Q000627:therapeutic use*; D002648:Child; D004562:Electrocardiography / Q000187:drug effects; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D006334:Heart Function Tests; D006801:Humans; D008297:Male; D013318:Stroke Volume / Q000187:drug effects; D016277:Ventricular Function, Left / Q000187:drug effects; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy*
D002331:Carnitine