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Abstract：
Background and Objectives: Cardiac magnetic resonance (CMR) imaging has become an essential instrument in the study of cardiomyopathies; it has recently been integrated into the diagnostic workflow for cardiac amyloidosis (CA) with remarkable results. An additional emerging role is the stratification of the arrhythmogenic risk by scar analysis and the possibility of merging these data with electro-anatomical maps. This is made possible by using a software (ADAS 3D, Galgo Medical, Barcelona, Spain) able to provide 3D heart models by detecting fibrosis along the whole thickness of the myocardial walls. Little is known regarding the applications of this software in the wide spectrum of cardiomyopathies and the potential benefits have yet to be discovered. In this study, we tried to apply the ADAS 3D in the context of CA. Materials and Methods: This study was a retrospectively analysis of consecutive CMR imaging of patients affected by CA that were treated in our center (Marche University Hospital). Wherever possible, the data were processed with the ADAS 3D software and analyzed for a correlation between the morphometric parameters and follow-up events. The outcome was a composite of all-cause mortality, unplanned cardiovascular hospitalizations, sustained ventricular arrhythmias (VAs), permanent reduction in left ventricular ejection fraction, and pacemaker implantation. The secondary outcomes were the need for a pacemaker implantation and sustained VAs. Results: A total of 14 patients were deemed eligible for the software analysis: 8 patients with wild type transthyretin CA, 5 with light chain CA, and 1 with transthyretin hereditary CA. The vast majority of imaging features was not related to the composite outcome, but atrial wall thickening displayed a significant association with both the primary (p = 0.003) and the secondary outcome of pacemaker implantation (p = 0.003). The software was able to differentiate between core zones and border zones of scars, with the latter being the most extensively represented in all patients. Interestingly, in a huge percentage of CMR images, the software identified the highest degree of core zone fibrosis among the epicardial layers and, in those patients, we found a higher incidence of the primary outcome, without reaching statistical significance (p = 0.18). Channels were found in the scar zones in a substantial percentage of patients without a clear correlation with follow-up events. Conclusions: CMR imaging plays a pivotal role in cardiovascular diagnostics. Our analysis shows the feasibility and applicability of such instrument for all types of CA. We could not only differentiate between different layers of scars, but we were also able to identify the presence of fibrosis channels among the different scar zones. None of the data derived from the ADAS 3D software seemed to be related to cardiac events in the follow-up, but this might be imputable to the restricted number of patients enrolled in the study.
摘要：
背景和目的：心脏磁共振（CMR）成像已成为研究心肌病的重要手段。它最近已被整合到心脏淀粉样变性（CA）的诊断工作流程中，并取得了显着成果。另一个新出现的作用是通过疤痕分析对心律失常风险进行分层，以及将这些数据与电解剖图合并的可能性。这是通过使用软件(ADAS3D，加尔戈医疗，巴塞罗那,西班牙）能够通过检测心肌壁整个厚度的纤维化来提供3D心脏模型。关于该软件在广泛的心肌病中的应用知之甚少，潜在的好处尚未发现。在这项研究中,我们尝试在CA的上下文中应用ADAS3D。材料和方法：本研究是对我们中心（马尔凯大学医院）治疗的CA患者的连续CMR成像的回顾性分析。只要有可能,使用ADAS3D软件处理数据,并分析形态测量参数与随访事件之间的相关性.结果是全因死亡率的综合结果，计划外的心血管住院,持续性室性心律失常（VA），左心室射血分数永久性降低,和起搏器植入。次要结果是需要起搏器植入和持续的VAs。结果：共有14例患者被认为符合软件分析的条件：8例患者患有野生型甲状腺素运载蛋白CA，5带轻链CA，1与转甲状腺素蛋白遗传性CA。绝大多数影像学特征与复合结局无关，但心房壁增厚与起搏器植入的主要结局(p=0.003)和次要结局(p=0.003)均有显著关联.该软件能够区分疤痕的核心区域和边界区域，后者是所有患者中最广泛的代表。有趣的是,在巨大比例的CMR图像中，该软件确定了心外膜中核心区纤维化程度最高，在这些患者中，我们发现主要结局的发生率较高，没有达到统计学意义（p=0.18）。在大部分患者的疤痕区域中发现了通道，与随访事件没有明显的相关性。结论：CMR成像在心血管诊断中起着关键作用。我们的分析表明了这种仪器对所有类型CA的可行性和适用性。我们不仅可以区分不同层的疤痕，但我们也能够确定不同瘢痕区之间是否存在纤维化通道.来自ADAS3D软件的数据似乎都与随访中的心脏事件无关。但这可能归因于纳入研究的患者数量有限.
