背景:心脏淀粉样变性越来越被认为是心血管疾病发病率和死亡率的重要因素。随着新疗法的出现,人们对使用心脏磁共振成像(CMR)预测心脏淀粉样变性患者的兴趣日益浓厚.在这篇系统综述和荟萃分析中,我们旨在研究心肌天然T1和T2以及细胞外体积(ECV)的预后意义.
方法:符合观察性队列研究或单组临床试验标准。MEDLINE,从各自的成立日期到2023年1月,对EMBASE和CENTRAL进行了系统搜索。没有根据发布日期做出排除,研究结果,或者学习语言。研究人群由淀粉样心肌病的成年患者(≥18岁)组成。所有研究都包括使用CMR,有或没有静脉注射钆对比剂,以评估心肌天然T1标测。T2映射,和ECV与预先指定的全因死亡率的主要结局相关。由两名独立的审阅者从符合条件的主要研究中提取数据,并使用随机效应模型通过逆方差方法进行汇总以进行荟萃分析。
结果:共审查了3852篇引文。最后九项研究包括955名患者(平均年龄65±10岁,32%女性,平均左心室射血分数(LVEF)59±12%和24%有NYHAIII或IV级症状)伴有心脏淀粉样变性[轻链淀粉样变性(AL)50%,甲状腺素运载蛋白淀粉样变性(ATTR)49%,其他1%]符合纳入条件,适用于数据提取。所有纳入的研究都是单中心的(七个使用1.5TMRI扫描仪,两个带有3.0TMRI扫描仪)并且在设计上是非随机的,随访时间为8~64个月(中位随访时间=25个月);320例患者在随访期间死亡,在所有研究中加权死亡率为33%。与AL淀粉样蛋白患者相比,ATTR淀粉样蛋白患者的平均左心室质量指数(LVMi)显着升高,(102±34g/m2对127±37g/m2,p=0.02)。N末端脑钠肽前体(NT-proBNP),肌钙蛋白T水平,平均原生T1值,ATTR淀粉样蛋白和AL淀粉样蛋白患者的ECV和T2值没有差异(均p>0.25)。总的来说,死亡率的危险比为1.33(95%CI=[1.10,1.60];p=0.003;I2=29%,)对于每60毫秒更高的T1时间,1.16(95%CI=[1.09,1.23],p<0.0001;I2=76%)ECV每增加3%,和5.23(95%CI=[2.27,12.02];p<0.0001;I2=0%),心肌-骨骼T2比率低于平均值(与高于平均值)。
结论:较高的固有T1时间和ECV,和较低的心肌与骨骼的T2比值,在CMR上,心脏淀粉样变性患者的死亡率更高。因此,使用CMR进行组织标测可以提供一种有用的非侵入性技术来监测疾病进展并确定心脏淀粉样变性患者的预后.
BACKGROUND: Cardiac amyloidosis is increasingly recognized as a significant contributor to cardiovascular morbidity and mortality. With the emergence of novel therapies, there is a growing interest in prognostication of patients with cardiac amyloidosis using cardiac magnetic resonance imaging (CMR). In this systematic review and meta-analysis, we aimed to examine the prognostic significance of myocardial native T1 and T2, and extracellular volume (ECV).
METHODS: Observational cohort studies or single arms of clinical trials were eligible. MEDLINE, EMBASE and CENTRAL were systematically searched from their respective dates of inception to January 2023. No exclusions were made based on date of publication, study outcomes, or study language. The study populations composed of adult patients (≥18 years old) with amyloid cardiomyopathy. All studies included the use of CMR with and without intravenous gadolinium contrast administration to assess myocardial native T1 mapping, T2 mapping, and ECV in association with the pre-specified primary outcome of all-cause mortality. Data were extracted from eligible primary studies by two independent reviewers and pooled via the inverse variance method using random effects models for meta-analysis.
RESULTS: A total of 3852 citations were reviewed. A final nine studies including a total of 955 patients (mean age 65 ± 10 years old, 32% female, mean left ventricular ejection fraction (LVEF) 59 ± 12% and 24% had NYHA class III or IV symptoms) with cardiac amyloidosis [light chain amyloidosis (AL) 50%, transthyretin amyloidosis (ATTR) 49%, other 1%] were eligible for inclusion and suitable for data extraction. All included studies were single centered (seven with 1.5 T MRI scanners, two with 3.0 T MRI scanners) and non-randomized in design, with follow-up spanning from 8 to 64 months (median follow-up = 25 months); 320 patients died during follow-up, rendering a weighted mortality rate of 33% across studies. Compared with patients with AL amyloid, patients with ATTR amyloid had significantly higher mean left ventricular mass index (LVMi) (102 ± 34 g/m2 vs 127 ± 37 g/m2, p = 0.02). N-terminal pro-brain natriuretic peptide (NT-proBNP), troponin T levels, mean native T1 values, ECV and T2 values did not differ between patients with ATTR amyloid and AL amyloid (all p > 0.25). Overall, the hazard ratios for mortality were 1.33 (95% CI = [1.10, 1.60]; p = 0.003; I2 = 29%) for every 60 ms higher T1 time, 1.16 (95% CI = [1.09, 1.23], p < 0.0001; I2 = 76%) for every 3% higher ECV, and 5.23 (95% CI = [2.27, 12.02]; p < 0.0001; I2 = 0%) for myocardial-to-skeletal T2 ratio below the mean (vs above the mean).
CONCLUSIONS: Higher native T1 time and ECV, and lower myocardial to skeletal T2 ratio, on CMR are associated with worse mortality in patients with cardiac amyloidosis. Therefore, tissue mapping using CMR may offer a useful non-invasive technique to monitor disease progression and determine prognosis in patients with cardiac amyloidosis.