بررسي عوارض قلبي و مرگ ومير ناشي از آن در بيماران بتاتالاسمي ماژور در 10 سال گذشته در بيمارستان حضرت علي اصغر(ع )

ASSESSMENT OF CARDIAC COMPLICATIONS AND MORTALITY RATE OF BETA THALASSEMIA MAJOR IN HAZRAT ALl ASGHAR CHILDREN HOSPITAL IN THE PAST TEN YEARS

Cardiac symptoms and premature death from cardiac causes are still major problem with beta thalassemia despite chelation therapy. Heart complications are the leading causes of mortality in the abscnce of effective iron chelation therapy. Many patients develop evidence of iron-induced myocardial damage with cardiac failure, cardiac arrhythmia, sudden death or death from progressive congestive failure. The characteristic lesion in the heart is caused by iron deposition in the myofibrils with myofibrillar fragmentation and diminished mitochondrial volume in myocyte. The basic cardiological assessment including I2-lead electrocardiogram, chest X-ray and echocardiogram is done from 7 years of age annually or more if it is needed. This study was undertaken on beta thalassemia patients who were under treatmcnt in Hazrat Ali Asghar Children Hospital in the past ten years. In total of 1069 cases, 16 patients(1.5°/0) died from severe cardiac impairment(CHF) and 19 patients with severe cardiac problems. who were treated with monitoring cardiac function and intensive chelation therapy, are alive and relatively well. 106(9.9%) cases are asyptomatic with moderate cardiac impairment and 985(92.1%) cases of thalasscmia are asymptomatic patients with normal heart condition.