Left ventricular function in thalassemia major: protective effect of deferoxamine.
OBJECTIVE
To test the hypothesis that chelation therapy with deferoxamine would prevent alterations in left ventricular systolic and diastolic function due to transfusional iron overload in patients with thalassemia major.


METHODS
A consecutive series of patients receiving chronic transfusional and chelation therapy were studied by two-dimensional and Doppler echocardiography.


METHODS
Primary clinic.


METHODS
Eight thalassemic patients (four men and four women), mean age 22 years (range 14 to 28) and seven age and sex matched control subjects.


METHODS
All patients had received transfusional therapy since birth, with mean annual load of red blood cells of 200 mL/kg. Iron chelation therapy with deferoxamine, using a subcutaneous infusion pump, was administered from age two years in the younger patients and from age 16 years in the two older cases. Doses were 25 mg/kg/day in children and 1.5 to 4 g per 12 h in adults to maintain ferritin blood levels at 1000 to 1500 ng/L.


RESULTS
No significant differences were found in the following Doppler diastolic indexes: isovolumic relaxation time, early flow velocity (E wave), late flow velocity (A wave), E:A ratio, rate of deceleration of flow velocity in early diastole (EF slope), flow velocity deceleration time and end-diastolic volume. Ejection fraction was similar in the two groups (59 +/- 7 versus 64 +/- 5%), but contractility, expressed as end-systolic pressure/end-systolic volume index, appeared slightly depressed (4.6 +/- 1 versus 6.7 +/- 0.8) in the thalassemic group.


CONCLUSIONS
Deferoxamine prevents alteration of left ventricular diastolic function in chronic transfusional therapy for thalassemia major. Depression of contractility, in spite of a normal ejection fraction, may be an early sign of worsening systolic performance, unavoidable even with chelation therapy.
Desideri
A
A
Cardiology and Transfusion Center, Este General Hospital, Padova, Italy.
Scattolin
G
G
Gabellini
A
A
Cavuto
F
F
Vanzelli
M
M
Formichi
M
M
Corbara
F
F
D000293:Adolescent; D000328:Adult; D001783:Blood Flow Velocity; D003676:Deferoxamine / Q000627:therapeutic use*; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D009200:Myocardial Contraction; D016277:Ventricular Function, Left; D017086:beta-Thalassemia / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000628:therapy
D003676:Deferoxamine