1st Cardiology Department, Aristotle University of Thessaloniki, AHEPA University Hospital, Thessaloniki, Greece.
1st Cardiology Department, Aristotle University of Thessaloniki, AHEPA University Hospital, Thessaloniki, Greece.
Hellenic J Cardiol. 2021 Nov-Dec;62(6):429-438. doi: 10.1016/j.hjc.2021.01.010. Epub 2021 Jan 29.
The current study evaluated the association of echocardiography, cardiac magnetic resonance (CMR), and ferritin data with 10-year survival in thalassemia patients.
Demographics, ferritin, echocardiography, and CMR parameters of stable consecutive thalassemia patients were prospectively collected.
In total, 75 patients (mean age 37 ± 11 years, 45% male) with thalassemia were included and dichotomized based on their survival status after a median follow-up period of 10.3 [9.6-10.9] years. Older age (HR: 1.071, p = 0.001), ferritin ≥2000 ng/ml (HR: 4.682, p = 0.007) and ≥1700 ng/ml (HR: 7.817, p = 0.002), elevated LV end-diastolic pressure (HR: 1.019, p = 0.044), TR Vmax >2.8 m/s (HR: 6.845, p = 0.005), and CMR T2∗ ≤20 msec (HR: 3.602, p = 0.043) and ≤34 msec (HR: 5.854, p = 0.026) were associated with increased all-cause mortality (primary endpoint). A baseline model including age was created and became more predictive of worse survival by adding TR Vmax >2.8 m/s instead of elevated LV end-diastolic pressure (C index 0.767 vs. 0.760, respectively), ferritin ≥1700 ng/ml instead of ≥2000 ng/ml (C index 0.890 vs. 0.807, respectively), or CMR T2∗≤34 msec instead of ≤20 msec (C index 0.845 vs. 0.839, respectively). Parameters associated with the combined endpoint of cardiac mortality/cardiac hospitalization (secondary endpoint) after adjusting for age were ferritin ≥1700 ng/ml (HR 3.770, p = 0.014), ratio E/A wave >2 (HR 3.565, p = 0.04), TR Vmax >2.8 m/s (HR 4.541, p = 0.049), CMR T2∗ ≤20 ms (HR 9.462, p = 0.001), and CMR T2∗ ≤34 ms (HR 11.735, p = 0.002). The model including age and T2∗ ≤34 ms instead of T2∗ ≤20 ms was more predictive of the secondary endpoint (C-index 0.844 vs 0.838, respectively).
In thalassemia patients, TR Vmax >2.8 m/s, ferritin ≥2000 ng/ml, and CMR T2∗ ≤20 ms were associated with worse long-term survival. In the current era of advanced chelation therapy, aiming for ferritin ≤1700 ng/ml and CMR T2∗ ≥34 ms may improve their prognosis.
本研究旨在评估超声心动图、心脏磁共振（CMR）和铁蛋白数据与地中海贫血患者 10 年生存率的关系。
前瞻性收集稳定的连续地中海贫血患者的人口统计学、铁蛋白、超声心动图和 CMR 参数。
共纳入 75 例（平均年龄 37±11 岁，45%为男性）地中海贫血患者，根据中位随访 10.3[9.6-10.9]年后的生存状态进行了分组。年龄较大（HR：1.071，p=0.001）、铁蛋白≥2000ng/ml（HR：4.682，p=0.007）和≥1700ng/ml（HR：7.817，p=0.002）、左室舒张末期压升高（HR：1.019，p=0.044）、TR Vmax＞2.8m/s（HR：6.845，p=0.005）和 CMR T2∗≤20msec（HR：3.602，p=0.043）和≤34msec（HR：5.854，p=0.026）与全因死亡率增加相关（主要终点）。创建了一个包含年龄的基线模型，并通过添加 TR Vmax＞2.8m/s 而不是升高的 LV 舒张末期压（指数分别为 0.767 与 0.760）、铁蛋白≥1700ng/ml 而不是≥2000ng/ml（指数分别为 0.890 与 0.807）或 CMR T2∗≤34msec 而不是≤20msec（指数分别为 0.845 与 0.839）来提高预测生存能力。在调整年龄后，与心脏死亡率/心脏住院率（次要终点）相关的参数为铁蛋白≥1700ng/ml（HR 3.770，p=0.014）、E/A 波比值＞2（HR 3.565，p=0.04）、TR Vmax＞2.8m/s（HR 4.541，p=0.049）、CMR T2∗≤20ms（HR 9.462，p=0.001）和 CMR T2∗≤34ms（HR 11.735，p=0.002）。包括年龄和 T2∗≤34ms 而不是 T2∗≤20ms 的模型对次要终点具有更好的预测能力（指数分别为 0.844 与 0.838）。
在地中海贫血患者中，TR Vmax＞2.8m/s、铁蛋白≥2000ng/ml 和 CMR T2∗≤20ms 与长期生存不良相关。在当前先进螯合治疗的时代，目标是铁蛋白≤1700ng/ml 和 CMR T2∗≥34ms 可能改善其预后。
