心肌固有 T2 测量可区分轻链和转甲状腺素蛋白心脏淀粉样变性并评估预后。
Myocardial native T2 measurement to differentiate light-chain and transthyretin cardiac amyloidosis and assess prognosis.
机构信息
Radiology Department, Henri Mondor Hospital, University Paris Est Créteil, Assistance Publique-Hôpitaux de Paris, 51 av Mal de Lattre de Tassigny, 94000, Créteil, France.
Cardiology Department, Henri Mondor Hospital, University Paris Est Créteil, Assistance Publique-Hôpitaux de Paris, Créteil, France.
出版信息
J Cardiovasc Magn Reson. 2018 Aug 16;20(1):58. doi: 10.1186/s12968-018-0478-3.
BACKGROUND
To assess the diagnostic and prognosis value of myocardial native T2 measurement in the distinction between Light-chain (AL) and Transthyretin (ATTR) cardiac amyloidosis (CA).
METHODS
Forty-four patients with CA (24 AL; 20 ATTR) and 40 healthy subjects underwent 1.5 T cardiovascular magnetic resonance (CMR). They all underwent T1 and T2 mapping (modified Look-Locker inversion recovery), cine and late gadolinium enhancement (LGE) imaging. The Query Amyloid Late Enhancement (QALE) score, myocardial native T2, T1 and extra cellular volume fraction (ECV) were calculated for all patients.
RESULTS
Of the 44 patients, 36 (82%) exhibited enhancement on LGE images. Mean QALE score of AL (7.9 ± 6) and ATTR (10.5 ± 5) patients were similar (p = 0.6). Myocardial native T2 was significantly (p < 0.0001) higher in AL (63.2 ± 4.7 ms) than in ATTR (56.2 ± 3.1 ms) patients, and both higher (p < 0.001) than healthy subjects (51.1 ± 3.1 ms). Myocardial native T2 was highly correlated with myocardial native T1 (Spearman's rho = 0.79; p < 0.001) and exhibited higher diagnostic performance than T1 to separate AL and ATTR patients: the area under curve (AUC) of T2 was 0.94 (95% CI: 0.86-1, p < 0.001) and the AUC of T1 was 0.77 (95% CI: 0.62-0.91, p = 0.03). Myocardial native T2 did not impact overall survival in patients (HR 1.03 (0.94-1.12); p = 0.53) in contrast to ECV that was the best predictor of outcome (HR 1.66 per 0.1 increase in ECV (1.24-2.22); p = 0.0006).
CONCLUSIONS
Myocardial native T2 significantly is increased in CA, especially in AL patients in comparison to ATTR patients. Myocardial native T2 does not impact survival in CA patients in contrast to ECV that was the best predictor of outcome.
TRIAL REGISTRATION
Trial Registration and unique number: CNIL cardio 1778041. Date of registration: 20 December 2012.
背景
评估心肌固有 T2 测量在区分轻链 (AL) 和转甲状腺素蛋白 (ATTR) 心脏淀粉样变性 (CA) 中的诊断和预后价值。
方法
44 例 CA 患者（24 例 AL；20 例 ATTR）和 40 例健康对照者接受 1.5 T 心血管磁共振（CMR）检查。所有患者均进行 T1 和 T2 mapping（改良 Look-Locker 反转恢复）、电影和晚期钆增强（LGE）成像。计算所有患者的 Query Amyloid Late Enhancement（QALE）评分、心肌固有 T2、T1 和细胞外容积分数（ECV）。
结果
44 例患者中，36 例（82%）LGE 图像增强。AL（7.9±6）和 ATTR（10.5±5）患者的平均 QALE 评分相似（p=0.6）。AL 患者的心肌固有 T2 明显高于 ATTR 患者（63.2±4.7 ms；p<0.0001），且均高于健康对照组（51.1±3.1 ms；p<0.001）。心肌固有 T2 与心肌固有 T1 高度相关（Spearman's rho=0.79；p<0.001），并具有比 T1 更高的诊断性能来区分 AL 和 ATTR 患者：T2 的 AUC 为 0.94（95%CI：0.86-1，p<0.001），T1 的 AUC 为 0.77（95%CI：0.62-0.91，p=0.03）。与 ECV 相比，心肌固有 T2 并未影响患者的总生存率（HR 1.03（0.94-1.12）；p=0.53），而 ECV 是最佳预后预测因子（每增加 0.1 ECV，HR 增加 1.66（1.24-2.22）；p=0.0006）。
结论
与 ATTR 患者相比，心肌固有 T2 在 CA 中明显升高，尤其是在 AL 患者中。与 ECV 相比，心肌固有 T2 对 CA 患者的生存没有影响，而 ECV 是最佳的预后预测因子。
试验注册
试验注册和唯一编号：CNIL cardio 1778041。注册日期：2012 年 12 月 20 日。
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