بررسی عوارض قلبی در بیماران مبتلا به تالاسمی ماژور در کاشان در سالهای 1378 لغایت 1379

ABSTRACT Due to the frequency of major thalassemia in our country and cardiac complications secondry to iron overload as one of the most important causes of death in these patients and the absence of any similar study in this region (Kashan city), we studied the cardiac complications in patients with major thalassemia in Kashan in 1999 and 2000. A prospective descriptive study was performed on seventeen patients with major thalassemia. All patients underwent a thorough noninvasive cardiovascular investigation including history taking, physical examination, electrocardiography (ECG), chest roentgenography (CXR) and complete two-dimensional, color M-Mode Doppler echocardiography. Arterial blood pressure and indices of diastolic function by Doppler echocardiography were measured in seventeen age and sex-matched healthy individuals as control group. The obtained data were recorded and standard deviation was calculated. P value less than 0.05 was considered as significant. A study was carried out on seventeen patients (including eleven male and six female) with the age range of 18 months to 25 years old (mean 14 years). Weakness and easy fatiguabiltiy were the most common complaints in the history of patients. In all patients arterial blood pressure was between the 5% and 25% for height, sex and age (significantly lower than the control group) ECG and cardiac size in CXR were normal in 82% and 76% of patients respectively. Abnormal findings in the ECG included left ventricular hypertrophy in two patients and first degree atrioventericular block (not present in the ECG of the same patient at six months ago) in one of the cases. Diastolic and systolic dysfunction were observed in 82% and 11% of cases respectively, by echocardiography. Diastolic dysfunction occurs earlier than systolic dysfunction in patients with major thalassemia and electrocardiography and chest X-Ray are not sensitive tools for early detection of diastolic dysfunction in these patients. Echocardiogarphy is the best noninvasive means for evaluation of ventricular function particularly diastolic function and is recommended for early detection of cardiac complications in these patients.