Prognostic significance of left ventricular diastolic indexes in beta-thalassemia major.
OBJECTIVE
To define the prognostic significance of left ventricular diastolic function in patients with beta-thalassemia major by noninvasive M-mode echocardiography and Doppler measurements.


METHODS
Cohort analytic study.


METHODS
A university hospital in Taipei, Taiwan.


METHODS
Forty-five patients (age range, 4 to 25 years) with transfusion-dependent beta-thalassemia major followed up for 5 years.


METHODS
None.


RESULTS
M-mode and Doppler echocardiography were used to determine the left systolic time interval; ejection fraction; left atrium emptying index; isovolumetric relaxation period; diastolic total period; the duration, slope, and height of the early diastolic flow-velocity peak; and the ratio between the heights of early and atrial diastolic flow-velocity peaks. All patients had normal systolic ventricular function initially, but impaired diastolic function developed, especially in the older age group who had more evidence of iron loading. In nine of those with abnormal diastolic total period (n = 10) and duration of the early diastolic flow-velocity peak (n = 12) values, regardless of age, congestive heart failure developed during the period of follow-up. Of these nine, four died within 2 years after the advent of heart failure and five remained compensated after anticongestive treatment and use of deferoxamine mesylate. The presence of an abnormal diastolic total period and duration of the early diastolic flow-velocity peak values correlated well with the prognosis.


CONCLUSIONS
Left ventricular diastolic filling variables by echocardiography are important predictors of the outcome of patients with transfusion-dependent beta-thalassemia major and could provide useful measures in determining the effects of medication during long-term follow-up.
Hou
J W
JW
Department of Pediatrics, National Taiwan University Hospital, Taipei.
Wu
M H
MH
Lin
K H
KH
Lue
H C
HC
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000627:therapeutic use; D003971:Diastole; D004452:Echocardiography / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008297:Male; D011379:Prognosis; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D013318:Stroke Volume; D013599:Systole; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000628:therapy
D003676:Deferoxamine