1. Heart & Thalassemia .
R.Miri,MD, Interventional Cardiologist

2. Heart & Thalassemia
Cardiac complications represent the primary cause of mortality / morbidity

3. Heart & Thalassemia iron is normally transported by transferrin
in iron overload, transferrin becomes completely saturated, so free iron enters the cardiomyocytes

4. Heart & Thalassemia
Inside the cardiomyocytes, iron is stored in 3 different forms:
ferritin
Hemosiderin
labile cellular iron
Labile cellular iron is the most accessible to iron chelators but also the most toxic form, inducing cardiomyocyte apoptosis and finally, cardiac dysfunction.

5. Heart & Thalassemia
The susceptibility to iron toxicity varies greatly among thalassemia cases
patients with the same iron load present with various severity of cardiac injury.

6. Heart & Thalassemia

7. Heart & Thalassemia
Early identification of ventricular dysfunction, before the appearance of symptoms, can alter the prognosis of these patients

8. Heart & Thalassemia
cardiomyopathy that progressively leads to heart failure and death.

9. Heart & Thalassemia Cardiomyopathy phenotypes:
Dilated phenotype, with left ventricular dilatation and impaired contractility(Viral Myocarditis)
Restrictive phenotype, with restrictive left ventricular filling, pulmonary hypertension, and right heart failure( Iron Overload). 

10. dilated cardiomyopathy
4.5% of cases developed a clinical picture consistent with acute myocarditis at a mean age of 15±3 years, because of their repetitive exposure to a significant, transfusion-associated viral burden

11. Restrictive cardiomyopathy
a) Due iron overload results from:
ineffective erythropoiesis
peripheral hemolysis
increased intestinal iron absorption
main cause is the repetitive blood transfusions
b) anemia / bone marrow expansion cause volume overload and increased cardiac output, with consequent development of eccentric ventricular hypertrophy

12. Heart & Thalassemia
Our study is an observational and cross sectional study including 145 patients with thalassemia major with no signs or symptoms of heart failure

13. Echo Measurment LV (end-diastolic / end-systolic) diameters & volumes
IVS & LV posterior wall thicknesses (end-diastolic / end-systolic)
LV ejection fraction
LA volume
Mitral early (E) & late (A) diastolic velocities
E/A ratio
isovolumetric relaxation time

15. LV diastolic function parameters
early diastolic wave (E wave)
late diastolic wave (A wave)
deceleration time of E wave (DT)
isovolumicrelaxation time (IVRT)
E wave to A wave (E/A) ratio

16. LV systolic function parametersEF
Sympsons Method

17. Echo Measurment
LA volume indexed by body surface area was significantly higher
suggests an increase in LVEDP, reflecting an alteration in diastolic function

18. independent predictor of
Echo Measurment
An study with 6,657 individuals showed that
LA volume / body surface area ratio ≥ 34 ml/m² is an
independent predictor of
death
heart failure
atrial fibrillation
ischemic stroke

19. LV diastolic function parameters
higher E/A ratio were suggestive of restrictive diastolic pattern and consequently stiff LV wall

20. LV diastolic function parameters

21. LV diastolic function parameters
the decreased DT of E wave related to the impaired relaxation of LV.
The IVRT was shortened which is the reflection of the impaired LV relaxation pattern most probably caused by iron overload stiffness of LV wall.

22. LV systolic function evaluation:
LV systolic posterior wall thickening was lower
Simpson method showed LV systolic dysfunction

23. Pulmonary Hypertension
Seen in 15% of the cases, evauated by TR
PH results from :
Hypercoagulability
endothelial dysfunction
reduce NO availability
elastic tissue disorders
lung tissue injury

24. Angiography In 13 of the 145 was done ,age:12-25 yr
Normal coronaries arteries despite of severe iron disposition.