Department of Therapeutics, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Research Unit of Radiology and Medical Imaging, 2nd Department of Radiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Curr Probl Cardiol. 2023 Apr;48(4):101573. doi: 10.1016/j.cpcardiol.2022.101573. Epub 2022 Dec 28.
Early diagnosis is crucial for the improvement of outcomes of patients with cardiac amyloidosis (CA). Emerging non-late gadolinium enhancement (LGE) based cardiac magnetic resonance (CMR) parameters may facilitate early identification of CA. We sought to investigate the diagnostic and prognostic value of T1, T2 mapping and extracellular volume (ECV) in CA. This single-center prospective analysis included 88 patients with CA, 33 patients with aortic stenosis (AS) and left ventricular hypertrophy (LVH), and 15 healthy controls who completed 3T cardiac MRI at the time of their diagnosis and were assessed with T1, T2 (modified Look-Locker inversion recovery), and ECV mapping of the heart and spleen. Echocardiographic, and biochemical parameters and clinical characteristics and outcomes were collected and analyzed. Of the patients with CA, 71 had light-chain (AL) and 17 had transthyretin (ATTR) amyloidosis. Native T1, native T2 and ECV were significantly higher in patients with CA compared to both patients with LVH-AS (P<0.001) and healthy controls (P<0.001). Good diagnostic accuracy was also demonstrated by measuring the area under the curve (AUC) of the receiver operating characteristic (ROC) curves for native T1 in the region of interest (ROI) (AUC=0.90), native T2 ROI (AUC=0.88), and ECV (AUC=0.90). Furthermore, native T1 ROI, native T2 ROI and ECV, correlated with both NT-proBNP levels and Mayo stage of patients (with AL). Spleen ECV was significantly increased in patients with AL versus ATTR amyloidosis (38.5 vs 30.5; P=0.004) and demonstrated good diagnostic accuracy in differentiating between the two types (AUC=0.79). Native T2 ROI was prognostic of mortality in AL CAwith a HR of 1.97 per 5 ms increase (P=0.001) and remained prognostic after adjustment for age, and Mayo stage. Non-LGE based CMR techniques correlated with established markers of disease and demonstrated good diagnostic accuracy, while native T2 ROI was also prognostic of mortality, thus reinforcing their use in the diagnosis and prognosis of CA.
早期诊断对于改善心脏淀粉样变（CA）患者的预后至关重要。新兴的基于钆延迟增强（LGE）的心脏磁共振（CMR）参数可能有助于早期识别 CA。我们旨在研究 T1、T2 映射和细胞外容积（ECV）在 CA 中的诊断和预后价值。这项单中心前瞻性分析纳入了 88 例 CA 患者、33 例主动脉瓣狭窄（AS）伴左心室肥厚（LVH）患者和 15 例健康对照者，他们在诊断时完成了 3T 心脏 MRI 检查，并进行了心脏和脾脏的 T1、T2（改良 Look-Locker 反转恢复）和 ECV 映射。收集并分析了超声心动图、生化参数以及临床特征和结局。88 例 CA 患者中，71 例为轻链（AL）淀粉样变性，17 例为转甲状腺素蛋白（ATTR）淀粉样变性。与 LVH-AS 患者（P<0.001）和健康对照者（P<0.001）相比，CA 患者的心脏 T1 本底值、T2 本底值和 ECV 明显更高。通过测量心脏 ROI（感兴趣区）T1 本底值（ROC 曲线下面积（AUC）=0.90）、T2 本底值 ROI（AUC=0.88）和 ECV（AUC=0.90）的 AUC 值，也显示出了良好的诊断准确性。此外，心脏 ROI 的 T1 本底值、T2 本底值和 ECV 与 NT-proBNP 水平和患者的 Mayo 分期（AL）均相关。与转甲状腺素蛋白（ATTR）淀粉样变性相比，AL 淀粉样变性患者的脾脏 ECV 明显升高（38.5 比 30.5；P=0.004），并在两种类型之间具有良好的诊断准确性（AUC=0.79）。T2 本底值 ROI 与 AL CA 的死亡率相关，每增加 5ms，风险比为 1.97（P=0.001），并且在调整年龄和 Mayo 分期后仍然具有预后意义。基于非 LGE 的 CMR 技术与疾病的既定标志物相关，具有良好的诊断准确性，而 T2 本底值 ROI 也是死亡率的预后因素，因此加强了它们在 CA 的诊断和预后中的应用。
