Heart Failure and Transplant Unit, Onassis Cardiac Surgery Center, Athens, Greece.
Hematology/Oncology Unit, First Department of Pediatrics, National and Kapodistrian University of Athens, Athens, Greece.
BMC Cardiovasc Disord. 2021 Feb 9;21(1):81. doi: 10.1186/s12872-021-01897-8.
Myocardial iron overload in patients with thalassemia major (TM) is one of the most important complications. The purpose of the study was to identify advanced echocardiography parameters for early identification of myocardial dysfunction during follow-up of patients with TM.
Forty TM patients who were 41 ± 5 years old were included in the study and divided into two groups according to cardiac magnetic resonance T2* results (Group 1: Τ2* > 25 ms, Group 2: Τ2* ≤ 25 ms). Liver T2* parameters were also measured. Conventional and deformational echocardiographic parameters were measured at baseline and approximately 2 years later.
Thirty-two patients had Τ2* = 34 ± 4 ms (Group 1), and 8 had Τ2* = 17 ± 9 ms (Group 2). Blood consumption was 185 ± 60 and 199 ± 37 ml/kg/yr (p = 0.64), and liver T2* was 4 ± 5 and 17 ± 21 ms (p = 0.01) in Groups 1 and 2, respectively. At baseline, Group 1 had better left ventricular global longitudinal strain (GLS) (- 22 ± 3 vs. - 18 ± 5, p = 0.01) and similar left ventricular ejection fraction (LVEF) (62 ± 5% vs. 58 ± 10%, p = 0.086) than Group 2. At the 28 ± 11-month follow-up, LVEF, GLS, and T2* values in Group 1 (63 ± 3%, - 21 ± 3%, 34 ± 4 ms) and Group 2 (56 ± 11%, - 17 ± 4%, 17 ± 9 ms) did not change significantly compared to their corresponding baseline values. In 8 patients from Group 1, a worsening (> 15%) in LS (p = 0.001) was detected during follow-up, with a marginal reduction in LVEF.
GLS seems to be an efficient echocardiographic parameter for detecting hemochromatosis-related cardiac dysfunction earlier than LVEF. It also seems to be affected by other factors (free radical oxygen, immunogenetic mechanisms or viral infections) in a minority of patients, underscoring the multifactorial etiology of cardiomyopathy.
铁过载是重型地中海贫血（TM）患者最重要的并发症之一。本研究的目的是确定先进的超声心动图参数，以便在 TM 患者的随访中早期识别心肌功能障碍。
研究纳入了 40 名年龄为 41±5 岁的 TM 患者，并根据心脏磁共振 T2结果将其分为两组（组 1：T2>25ms，组 2：T2*≤25ms）。还测量了肝脏 T2*参数。在基线和大约 2 年后测量常规和变形超声心动图参数。
32 名患者的 T2*=34±4ms（组 1），8 名患者的 T2*=17±9ms（组 2）。组 1 和组 2 的血液消耗分别为 185±60 和 199±37ml/kg/yr（p=0.64），肝脏 T2分别为 4±5 和 17±21ms（p=0.01）。在基线时，组 1 的左心室整体纵向应变（GLS）更好（-22±3%比-18±5%，p=0.01），左心室射血分数（LVEF）相似（62±5%比 58±10%，p=0.086）。在 28±11 个月的随访中，组 1（63±3%，-21±3%，34±4ms）和组 2（56±11%，-17±4%，17±9ms）的 LVEF、GLS 和 T2值与基线值相比均无显著变化。在组 1 的 8 名患者中，在随访期间检测到 LS（p=0.001）恶化（>15%），LVEF 略有下降。
GLS 似乎是一种有效的超声心动图参数，可比 LVEF 更早地检测到与血色素沉着症相关的心脏功能障碍。在少数患者中，它似乎也受到其他因素（自由基氧、免疫遗传机制或病毒感染）的影响，这突显了心肌病的多因素病因。
