Myocardial performance index in pediatric β-thalassemia major

الملخص EN

Background: Despite advancement in treatment, cardiac dysfunction remains to be the leading cause of death in patients with 0-thalassemia major.

The pathophysiology of cardiac disease in 0 -thalassemia is complex and may be related to many causes.

Aims: Determine the thalassemia-induced changes of myocardial performance index(MPI).

Methods: 27 patients with 0 -thalassemia major as well as 18 normal subjects were included , clinically evaluated and echocardiographically examined using two dimensional (2-D)-, M-mode, color flow mapping and spectral Doppler echo.

MPI was calculated by dividing the sum of isovolumic contraction and relaxation times by the left ventricular ejection time (McMahon et al., 2004 and Spences et alv 2004).

Investigations included serum ferritin and screening for transfusion-induced hepatitis B and C.

Results: MPI was significantly higher in cases versus controls (0.47 + 0.12 vs 0.39 ± 0.07 , p = 0.01) unlike left ventricular ejection fraction & fractional shortening (EF& FS) which were normal in patients vs controls (66.9 + 5.8 and 36.9 ± 4.8 vs 65.7 ± 6.2 and 35.9 + 4.9 %, P > 0.05) .The MPI correlated with the serum ferritin ( r = 0.49, p = 0.01).

Conclusion: Systolic and diastolic myocardial dysfunction are present in patients with p-thalassemia major and this is better detected by calculating the MPI rather than by measuring the traditional indices of LV function Thalassemia -induced myocardial dysfunction correlates positively with serum ferritin.