Heart involvement in transfusion-dependent betathalassemia with conventional echocardiography

Background: The most important prognostic factor in transfusion-dependent betathalassemia is cardiac involvement which is usually evaluated with echocardiography. Methods: In this cross-sectional study (April 2011 to April 2012), conventional echocardiography was used to assess myocardial performance and valvular involvement (through transvalvular Doppler study) for right and left heart abnormalities in transfusiondependent beta-thalassemia. Results: Among the 60 patients, 43 cases had heart problems, 26 (43.3%) of them had left myocardial dysfunction and 11 (18.3%) of them had right myocardial dysfunction, 3 cases had both RV and LV myocardial dysfunction, (based on LVMPI & RVMPI, respectively). In patients with right myocardial dysfunction, 4 cases had pulmonary hypertension (PH) and 3 had both sided myocardial dysfunction. LVMPI and RVMPI significantly increased in patients with cardiac involvement (p<0.001). Serum ferritin levels in patients with and without cardiac involvement were 2427±1788 ng/ml and 1573±592 ng/ml, respectively (P=0.008). All 4 patients who had PH, had been splenectomized. In splenectomized and nonsplenectomized patients, LVMPI was 0.37±0.11 and 0.38±0.1 (P=0.589), RVMPI was 0.3±0.07 and 0.25±0.39 (P=0.004), and TR gradient (TRG) was 28±11.8 mmHg and 19.7±5.2 mmHg (P=0.033), respectively. Mean ferritin level in patients with a history of splenectomy (n=31), was 2525±1968 ng/ml and in patients without the history of splenectomy (n=29) was 1821±947 ng/ml (P=0.082). Conclusion: In addition to left-sided heart involvement, conventional echocardiography revealed right-sided heart involvement in transfusion-dependent thalassemia patients which did not correlate with serum ferritin level in splenectomized patients.