Meloni Antonella, Pistoia Laura, Gamberini Maria Rita, Cuccia Liana, Lisi Roberto, Cecinati Valerio, Ricchi Paolo, Gerardi Calogera, Restaino Gennaro, Righi Riccardo, Positano Vincenzo, Cademartiri Filippo
Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Unità Operativa Complessa Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Diagnostics (Basel). 2023 Feb 26;13(5):890. doi: 10.3390/diagnostics13050890.
We assessed the prognostic value of multiparametric cardiovascular magnetic resonance (CMR) in predicting death from heart failure (HF) in thalassemia major (TM). We considered 1398 white TM patients (30.8 ± 8.9 years, 725 women) without a history of HF at baseline CMR, which was performed within the Myocardial Iron Overload in Thalassemia (MIOT) network. Iron overload was quantified by using the T2* technique, and biventricular function was determined with cine images. Late gadolinium enhancement (LGE) images were acquired to detect replacement myocardial fibrosis. During a mean follow-up of 4.83 ± 2.05 years, 49.1% of the patients changed the chelation regimen at least once; these patients were more likely to have significant myocardial iron overload (MIO) than patients who maintained the same regimen. Twelve (1.0%) patients died from HF. Significant MIO, ventricular dysfunction, ventricular dilation, and replacement myocardial fibrosis were identified as significant univariate prognosticators. Based on the presence of the four CMR predictors of HF death, patients were divided into three subgroups. Patients having all four markers had a significantly higher risk of dying for HF than patients without markers (hazard ratio (HR) = 89.93; 95%CI = 5.62-1439.46; = 0.001) or with one to three CMR markers (HR = 12.69; 95%CI = 1.60-100.36; = 0.016). Our findings promote the exploitation of the multiparametric potential of CMR, including LGE, for better risk stratification for TM patients.
我们评估了多参数心血管磁共振成像（CMR）在预测重型地中海贫血（TM）患者心力衰竭（HF）死亡方面的预后价值。我们纳入了1398例白人TM患者（年龄30.8±8.9岁，女性725例），这些患者在基线CMR检查时无HF病史，CMR检查在“地中海贫血心肌铁过载（MIOT）”网络内进行。采用T2*技术对铁过载进行定量分析，利用电影图像测定双心室功能。采集延迟钆增强（LGE）图像以检测替代性心肌纤维化。在平均4.83±2.05年的随访期间，49.1%的患者至少改变过一次螯合治疗方案；与维持相同治疗方案的患者相比，这些患者更有可能存在显著的心肌铁过载（MIO）。12例（1.0%）患者死于HF。显著的MIO、心室功能障碍、心室扩张和替代性心肌纤维化被确定为显著的单因素预后指标。根据HF死亡的四个CMR预测指标的存在情况，将患者分为三个亚组。具有所有四个指标的患者死于HF的风险显著高于无指标的患者（风险比（HR）=89.93；95%置信区间（CI）=5.62 - 1439.46；P = 0.001）或具有一至三个CMR指标的患者（HR = 12.69；95%CI = 1.60 - 100.36；P = 0.016）。我们的研究结果促进了对CMR多参数潜力（包括LGE）的利用，以便为TM患者进行更好的风险分层。
