Esfahani Hossein, Tanasan Asadolah, Rezanejad Mina, Torabian Saadat
Department of Pediatric Oncology, Besat Hospital, Hamadan University of Medical Sciences, Hamadan, Iran.
Department of Pediatrics, Besat Hospital, Hamadan University of Medical Sciences, Hamadan, Iran.
Caspian J Intern Med. 2021 Apr;12(3):243-248. doi: 10.22088/cjim.12.3.243.
The most important prognostic factor in transfusion-dependent beta-thalassemia is cardiac involvement which is usually evaluated with echocardiography.
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 transfusion-dependent beta-thalassemia.
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 non-splenectomized 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).
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.
输血依赖型β地中海贫血最重要的预后因素是心脏受累情况，通常通过超声心动图进行评估。
在这项横断面研究（2011年4月至2012年4月）中，采用传统超声心动图评估输血依赖型β地中海贫血患者右心和左心异常的心肌功能及瓣膜受累情况（通过跨瓣膜多普勒研究）。
60例患者中，43例存在心脏问题，其中26例（43.3%）有左心肌功能障碍，11例（18.3%）有右心肌功能障碍，3例同时存在右心室和左心室心肌功能障碍（分别基于左心室心肌性能指数和右心室心肌性能指数）。在右心肌功能障碍患者中，4例有肺动脉高压（PH），3例同时存在双侧心肌功能障碍。心脏受累患者的左心室心肌性能指数和右心室心肌性能指数显著升高（p<0.001）。有心脏受累和无心脏受累患者的血清铁蛋白水平分别为2427±1788 ng/ml和1573±592 ng/ml（P=0.008）。所有4例有肺动脉高压的患者均接受了脾切除术。在脾切除和未脾切除患者中，左心室心肌性能指数分别为0.37±0.11和0.38±0.1（P=0.589），右心室心肌性能指数分别为0.3±0.07和0.25±0.39（P=0.004），三尖瓣反流梯度分别为28±11.8 mmHg和19.7±5.2 mmHg（P=0.033）。有脾切除史患者（n=31）的平均铁蛋白水平为2525±1968 ng/ml，无脾切除史患者（n=29）的平均铁蛋白水平为1821±947 ng/ml（P=0.082）。
除了左侧心脏受累外，传统超声心动图显示输血依赖型地中海贫血患者右侧心脏也受累，且在脾切除患者中与血清铁蛋白水平无关。
