2D speckle tracking and atrial natriuretic peptide for assessment of the effect of carnitine deficiency on myocardial function in beta-thalassemia major patients / Antoine Fakhry Abdelmassih ; Supervised Hala Mounir Agha , Ornella Milanesi , Reem Ibrahim Esmail

Thesis (Ph.D.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Introduction: Heart disease remains the major cause of morbidity and mortality in Thalassemic patients. Multiple pathologies have been suggested in the development of cardiac dysfunction in Thalassemic patients including : iron overload in the heart1, leading to RV diastolic then LV systolic dysfunction , chronic anemia and the tissue hypoxia it induces together with Carnitine deficiency impair free fatty acid oxidation and ATP production in myocardial cells.. Because congestive heart failure is the main cause of death in these patients, early recognition of cardiac dysfunction may be useful in modifying the therapy. 2D and 3D STE and BNP may be a promising tool for such a purpose. Methods: Thirty-two Thalassemic patients with a mean age of 18.1± 7.03 years and 29 aged matched healthy control subjects have been included in the study. Patients have been recruited from pediatric hematology clinics in both Cairo University, Egypt (n=16) and Padova University, Italy (n=16). Conventional, TDI as well as 2D and 3D-STE was performed to all patients and control subjects in addition to myocardial relaxometry T2* by cardiac MRI and serum assays of Carnitine and BNP

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