بررسي شيوع عوارض قلبي اكتسابي ناشي از بيماري بتاتالاسمي ماژور در بيماران مراجعه كننده به مركز تالاسمي اهواز

Evaluation of acquired cardiac complications in major p-thalassemic patients referred to Ahvaz thalassemia center

Background and Objective: To evaluate cardiac status in patients with thalassemia major. Subjects and Methods: The investigation included thorough history taking, clinical examination, electrocardiography, chest radiography and echocardiography. One hundred and twenty young adults with /? thalassemia, following an adequate transfusional and chelation treatment regimen, without clinical signs of cardiopulmonary involvement. Results: 63 patients were female and 57 patients them were male. Mean age of the were 16.6 ± 6 years (range 5-37 yr). Abnormal findings in ECG were found in 35.8% that the most common were nonspecific T wave and ST segment changes. Chest radiograph revealed increased cardiothoracic ratio in 30% of patients. Precardial effusion reported in 2.5%. No history of precarditis was reported in any patient. Mitral, tricuspid and pulmonary valve regurgitations were observed in 52%, 60% and 20% respectively. None of the patients had frank congestive heart failure. Ejection fraction and fractional shortening of the patients were 55.2% ±5 and 31% ±5 respectively. E/A <1 [early (E) and late (A) transmitral and transtricuspid peak flow velocities] were seen in 2.5% and 4.2% respectively. Pulmonary hypertension (PHT; defined as pulmonary artery pressure > 30 mmHg) was found in 8.3% of the patients, aged 19±8 yr. PHT correlated positively with irregular use of desferal (P<0.001).Age correlated negatively with ejection fraction (PcO.OOl), fractional shortening (P<0.05), mitral E/A (p<0.003) and tricuspid E/A (p < 0.014). In patients with abnormal ECG and CXR; ejection fraction reduced significantly (P < 0.004). Left ventricular (LV) systolic dysfunction was encountered in 23.3% of patients, aged 20 ± 5 years. Restrictive LV filling was suggestive in 32% of patients. Conclusion: Despite of regular usage of desferal and standard transfusion, there is also chance of cardiac complications in thalassemics. Echocardiographic examination appears a tool more reliable than clinical, electrocardiographic and/or chest X ray examination in assessment of young adult patient with thalassemia major. At present the most powerful predictive information includes a patientʹs transfusion record, serial serum ferritin levels, and compliance to a chelation regimen