Division of Cardiology, Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.
Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
BMC Cardiovasc Disord. 2019 Nov 6;19(1):245. doi: 10.1186/s12872-019-1235-8.
The leading cause of mortality of thalassemia major patients is iron overload cardiomyopathy. Early diagnosis with searching for left ventricular diastolic dysfunction before the systolic dysfunction ensued might yield better prognosis. This study aimed to define the prevalence of the left ventricular diastolic dysfunction (LVDD) in thalassemia major patients with normal left ventricular systolic function and the associated factors.
Adult thalassemia major patients with normal left ventricular systolic function who were referred for cardiac T2* at Siriraj Hospital - Thailand's largest national tertiary referral center - during the October 2014 to January 2017 study period. Left ventricular diastolic function was defined by mitral valve filling parameters and left atrial volume index using CMR. Patients with moderate to severe valvular heart disease, pericardial disease, or incomplete data were excluded. Baseline characteristics, comorbid diseases, current medication, and laboratory results were recorded and analyzed.
One hundred and sixteen patients were included, with a mean age of 27.5 ± 13.5 years, 57.8% were female, and 87.9% were transfusion dependent. Proportions of homozygous beta-thalassemia and beta-thalassemia hemoglobin E were 12.1 and 87.9%, respectively. The baseline hematocrit was 26.3 ± 3.3%. The prevalence of LVDD was 20.7% (95% CI: 13.7-29.2%). Cardiac T2* was abnormal in 7.8% (95% CI: 3.6-14.2%). Multivariate analysis revealed age, body surface area, homozygous beta-thalassemia, splenectomy, heart rate, and diastolic blood pressure to be significantly associated with LVDD.
LVDD already exists from the early stages of the disease before the abnormal heart T2 * is detected. Homozygous beta-thalassemia and splenectomy were strong predictors of LVDD. These data may increase awareness of the disease, especially in the high risk groups.
重型地中海贫血患者的主要死亡原因是铁过载性心肌病。在收缩功能障碍之前，通过寻找左心室舒张功能障碍进行早期诊断，可能会获得更好的预后。本研究旨在确定左心室收缩功能正常的重型地中海贫血患者中左心室舒张功能障碍（LVDD）的患病率及其相关因素。
本研究纳入了 2014 年 10 月至 2017 年 1 月期间在泰国最大的国家三级转诊中心——诗里拉吉医院接受心脏 T2*检查的左心室收缩功能正常的成年重型地中海贫血患者。使用 CMR 评估二尖瓣充盈参数和左心房容积指数来定义左心室舒张功能。排除患有中度至重度瓣膜性心脏病、心包疾病或数据不完整的患者。记录并分析患者的基线特征、合并症、当前用药和实验室结果。
共纳入 116 例患者，平均年龄为 27.5±13.5 岁，57.8%为女性，87.9%依赖输血。纯合子β-地中海贫血和β-地中海贫血血红蛋白 E 的比例分别为 12.1%和 87.9%。基线血细胞比容为 26.3±3.3%。LVDD 的患病率为 20.7%（95%CI：13.7-29.2%）。T2*异常的患者占 7.8%（95%CI：3.6-14.2%）。多变量分析显示年龄、体表面积、纯合子β-地中海贫血、脾切除术、心率和舒张压与 LVDD 显著相关。
在异常的心脏 T2*被检测到之前，LVDD 已经存在于疾病的早期阶段。纯合子β-地中海贫血和脾切除术是 LVDD 的强烈预测因素。这些数据可能会提高对该疾病的认识，特别是在高危人群中。
