Isa Tafreshi Roya, Radgoodarzi Mohammad, Arjmandi Rafsanjani Kadijeh, Soheilipour Fahimeh
Department of Pediatric Cardiology, Ali Asghar Children's Hospital, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Front Pediatr. 2022 Jun 17;10:774528. doi: 10.3389/fped.2022.774528. eCollection 2022.
Cardiac complications are important causes of morbidity in patients with thalassemia intermedia (TI). We aimed to assess left ventricular (LV) function, using new tissue Doppler imaging (TDI) indices, in order to diagnose early ventricular impairment in asymptomatic children and adolescence with the TI.
We investigated possible differences in echocardiographic systolic and diastolic parameters between a population of 28 asymptomatic patients (mean age, 13.6 ± 5.7 years) and 35 age-matched healthy control members. All of them underwent 2-D, pulsed Doppler, and tissue Doppler echocardiographic studies for the assessment of the LV mass, Trans-mitral velocities, mitral annular systolic and diastolic velocities, myocardial performance index (MPI), and myocardial acceleration during isovolumic contraction (IVA). The cardiac iron load was estimated by magnetic resonance imaging T2*.
Left ventricular hypertrophy (LVH) was found in 13 (46.4%) patients. We found significantly reduced TDI-derived peak systolic myocardial velocity (s') in patients, whereas no significant difference was identified between the patients and control group members when the IVA was compared. The ratio of peak mitral inflow velocity to annular early diastolic velocity (E/e') of the mitral valve as an index of the diastolic function was significantly higher in patients (9 ± 1 vs. 6 ± 1, < 0.05). Choosing a TDI-derived MPI > 0.33 as a cutoff point, the global LV dysfunction was detected with a sensitivity of 78% and a specificity of 80%. The patients with LVH significantly exhibited higher values of TDI-MPI and lower values of s' velocity and IVA when compared against the subjects with normal LV mass.
Subtle LV systolic and diastolic dysfunction develops early in young patients with the TI who have normal cardiac iron concentration. Moreover, LV remodeling as a main cardiac adaptive response plays a principal role in developing myocardial impairment.
心脏并发症是中间型地中海贫血（TI）患者发病的重要原因。我们旨在使用新的组织多普勒成像（TDI）指标评估左心室（LV）功能，以便在无症状的TI儿童和青少年中早期诊断心室功能损害。
我们调查了28例无症状患者（平均年龄13.6±5.7岁）和35例年龄匹配的健康对照者之间超声心动图收缩和舒张参数的可能差异。他们均接受二维、脉冲多普勒和组织多普勒超声心动图检查，以评估左心室质量、跨二尖瓣速度、二尖瓣环收缩和舒张速度、心肌性能指数（MPI）以及等容收缩期心肌加速度（IVA）。通过磁共振成像T2*估计心脏铁负荷。
13例（46.4%）患者发现左心室肥厚（LVH）。我们发现患者中TDI衍生的收缩期心肌峰值速度（s'）显著降低，而比较IVA时患者与对照组之间未发现显著差异。作为舒张功能指标的二尖瓣峰值流入速度与二尖瓣环舒张早期速度之比（E/e'）在患者中显著更高（9±1对6±1，<0.05）。选择TDI衍生的MPI>0.33作为截断点，检测到整体左心室功能障碍的敏感性为78%，特异性为80%。与左心室质量正常的受试者相比，LVH患者的TDI-MPI值显著更高，s'速度和IVA值更低。
心脏铁浓度正常的TI年轻患者早期会出现轻微的左心室收缩和舒张功能障碍。此外，作为主要心脏适应性反应的左心室重构在心肌损害的发生中起主要作用。
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