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Analysis of Cardiac Metabolic Remodeling in Heart Failure Using Nuclear Medicine and Its Application: Japanese Society of Nuclear Cardiology Award. 心衰患者心脏代谢重构的核医学分析及其应用:日本核心脏病学会奖。
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00112
Takao Kato

Heart failure is associated with a significant change in the energy metabolism of the heart. We aimed to elucidate the altered energetics during the progression of heart failure. We used radioactive metabolic tracers to assess the substrate uptake. In a rat model of heart failure, the glucose uptake increased significantly at the stage of left ventricular hypertrophy, whereas the uptake of fatty acids decreased at the stage of heart failure, with decreased energy reserve during the transition of cardiac hypertrophy to failure. Metabolic modulator which enhances glucose oxidation ameliorated the decrease in cardiac function. We also validated the close correlation with mitochondrial membrane potentials and 99mtechnetium sestamibi (99mTc-MIBI) in vivo and at the organ level. The retention of 99mTc-MIBI signals was correlated with the severity of heart failure. Nuclear medicine is a powerful tool to understand the mechanism of cardiac remodeling in heart failure.

心力衰竭与心脏能量代谢的显著变化有关。我们的目的是阐明心力衰竭进展过程中能量学的改变。我们使用放射性代谢示踪剂来评估底物摄取。在心力衰竭大鼠模型中,左心室肥厚阶段葡萄糖摄取显著增加,而脂肪酸摄取在心力衰竭阶段减少,在心脏肥厚向心力衰竭过渡期间能量储备减少。代谢调节剂增强葡萄糖氧化,改善心功能下降。我们还在体内和器官水平上验证了线粒体膜电位和99mTc-MIBI之间的密切相关性。99mTc-MIBI信号的保留与心力衰竭的严重程度相关。核医学是了解心力衰竭心脏重构机制的有力工具。
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引用次数: 0
Simulation Study of High-sensitivity Cardiac-dedicated PET Systems with Different Geometries. 不同几何形状高灵敏度心脏专用PET系统的仿真研究。
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00114
Go Akamatsu, Hideaki Tashima, Yuma Iwao, Miwako Takahashi, Eiji Yoshida, Taiga Yamaya

Noninvasive quantification of myocardial blood flow with PET is a vital tool for detecting and monitoring of coronary artery disease. However, current standard cylindrical PET scanners are not optimized for cardiac imaging because they are designed mainly for whole-body imaging. In this study, we proposed two compact geometries, the elliptical geometry and the D-shape geometry, for cardiac-dedicated PET systems. We then evaluated their performance compared with a whole-body-size cylindrical geometry by using the Geant4 Monte Carlo simulation toolkit. In the simulation, an elliptical water phantom was scanned for 10-sec, and we calculated the sensitivity and the noise-equivalent count rate (NECR). Subsequently, a digital chest phantom was scanned for 30-sec and the coincidence data were reconstructed by in-house image reconstruction software. We evaluated the image noise in the liver region and the contrast recoveries in the heart region. Even with the limited number of detectors, the proposed compact geometries showed higher sensitivity than the whole-body geometry. The D-shape geometry achieved 47% higher NECR and 44% lower image noise compared with the whole-body cylindrical geometry. However, the contrasts in the hot area obtained by the proposed compact geometries were not as good as that obtained by the whole-body cylindrical geometry. There was no considerable difference in image quality between the elliptical geometry and the D-shape geometry. In conclusion, the compact geometries we have proposed are promising designs for a high-sensitivity and low-cost cardiac-dedicated PET system. A further study using a defect phantom model is required to evaluate the contrast of cold areas.

PET无创定量心肌血流是检测和监测冠状动脉疾病的重要工具。然而,目前标准的圆柱形PET扫描仪并没有优化心脏成像,因为它们主要是为全身成像设计的。在这项研究中,我们提出了两种紧凑的几何形状,椭圆几何形状和d形几何形状,用于心脏专用PET系统。然后,我们通过使用Geant4蒙特卡罗模拟工具包,将它们的性能与全身尺寸的圆柱形几何进行比较。在模拟中,我们对一个椭圆水影进行了10秒的扫描,并计算了灵敏度和噪声等效计数率(NECR)。随后,对数字胸影扫描30秒,并通过内部图像重建软件重建吻合数据。我们评估了肝脏区域的图像噪声和心脏区域的对比度恢复。即使在探测器数量有限的情况下,所提出的紧凑几何结构也比全身几何结构显示出更高的灵敏度。与全身圆柱形几何相比,d形几何的NECR提高了47%,图像噪声降低了44%。然而,所提出的紧凑几何形状所获得的热区对比度不如全身圆柱形几何形状所获得的对比度好。椭圆几何和d形几何在图像质量上没有显著差异。总之,我们提出的紧凑的几何形状是一种高灵敏度和低成本的心脏专用PET系统的有希望的设计。需要进一步研究使用缺陷模体模型来评估寒冷地区的对比度。
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引用次数: 0
Sample Size Considerations: Basics for Preparing Clinical or Basic Research. 样本量考虑:准备临床或基础研究的基础。
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00122
David N Williams, Kathryn A Williams

Background: Sample size estimation is an important and integral part of a research protocol. While "how large a sample?" is a simple question, the answer is only meaningful within the context of the research question. Methods: Clear definitions of the variable of interest and target population parameters are key to estimating sample size. In turn the sample must be sized such that it can accurately detect the 'effect' of interest, adequately represent the target population and maintain maximum design efficiency. Four basic pieces of information are utilized in most sample estimation across all clinical research: significance level, power, magnitude of effect and variability of the variable of interest. Results/Discussion: Preliminary determination of these will greatly facilitate work with a biostatistician or a computer application to create a sample size estimation. While applications can support relatively simple sample size calculations consultation with a biostatistician is recommended.

背景:样本量估计是研究方案的重要组成部分。虽然“样本有多大?”是一个简单的问题,但答案只有在研究问题的背景下才有意义。方法:明确兴趣变量和目标总体参数的定义是估计样本量的关键。反过来,样本的大小必须能够准确地检测到感兴趣的“效应”,充分代表目标人群,并保持最大的设计效率。在所有临床研究中,大多数样本估计使用了四个基本信息:显著性水平、功率、效应大小和感兴趣变量的可变性。结果/讨论:这些的初步确定将极大地促进生物统计学家或计算机应用程序创建样本大小估计的工作。虽然应用程序可以支持相对简单的样本大小计算,但建议咨询生物统计学家。
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引用次数: 0
Positive Myocardial Uptake of Bone Scintigraphic Agents Associated with Cardiac Amyloidosis: Frequency of Positive Uptake Data Based on Daily Clinical Practice. 与心脏淀粉样变性相关的骨显像药物心肌摄取阳性:基于日常临床实践的阳性摄取频率数据。
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00121
Shigeru Fukuzawa, Shinichi Okino, Hikaru Ishiwaki, Yo Iwata, Takashi Uchiyama, Nobuyuki Kuroiwa, Norikiyo Oka, Noeru Shibayama, Masayuki Inagaki

Objective: The bone scan plays an important role for detecting number of conditions relating to bones, including: bone cancer or bone metastasis, bone inflammation. Extraosseous uptake, in particular, myocardial uptake, was observed in some patients examined with the bone scans. Positive uptake of 99mTc-labeled bone radiotracers is associated with cardiac amyloidosis. However, the frequency and cause of positive cardiac 99mTc-MDP uptake have not been fully studied. In this regard, the aim of this study was to assess the frequency and characteristics of patients with high myocardi-al uptake of bone scintigraphy in daily clinical practice setting. Methods: We retrospectively analyzed 4180 bone scintigraphies performed in daily clinical practice during 7-years period. The intensity of the myocardial uptake was graded based on a visual scale ranging from 0 to 3 points. Score 0 indicates the absence of uptake. Score 1 defined uptake less than that of bone (referred to as the adjacent rib). Uptake similar to that of bone was classified score 2. Score 3 was defined as uptake greater than that of reference bone. Positive myocardial uptake included a visual score 2 or 3. Result: Positive 99mTc-MDP myocardial uptake occurred in 12 patients among 4180 patients (0.3%). 7 of 12 positive scan patients were consistent with amyloidosis confirmed by biopsy. In these patients, the mean age was 75.6 ± 5.2 years old. Ten cases showed biventricular uptake and 2 showed LV uptake only. Conclusion: Positive cardiac uptake of bone scintigraphic agents was present in 0.3% of bone scintigraphies in a clinical practice setting. This may be a sign of cardiac amyloidosis involvement which may give the presence of extraosseous bone tracer uptake its own importance and a new role.

目的:骨扫描在检测骨相关疾病中发挥重要作用,包括:骨癌或骨转移、骨炎症。在骨扫描检查的一些患者中观察到骨外摄取,特别是心肌摄取。99mtc标记的骨放射性示踪剂阳性摄取与心脏淀粉样变性有关。然而,心脏99mTc-MDP摄取阳性的频率和原因尚未得到充分研究。在这方面,本研究的目的是评估在日常临床实践中骨显像高心肌摄取患者的频率和特征。方法:回顾性分析7年来在临床实践中进行的4180例骨显像。心肌摄取的强度根据视觉评分从0到3分进行分级。0分表示没有摄取。评分1定义摄取少于骨(指相邻肋骨)。摄取与骨相似,评分2分。评分3定义为摄取大于参考骨。心肌摄取阳性包括视觉评分2或3分。结果:4180例患者中99mTc-MDP心肌摄取阳性12例(0.3%)。12例扫描阳性患者中有7例与活检证实的淀粉样变一致。患者平均年龄为75.6±5.2岁。双室摄取10例,左室摄取2例。结论:在临床实践中,0.3%的骨显像中出现了骨显像药物的心脏摄取阳性。这可能是心脏淀粉样变性的一个征象,这可能使骨外骨示踪剂摄取的存在具有其自身的重要性和新的作用。
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引用次数: 1
Comparing and Contrasting Guidelines for the Management of Cardiac Sarcoidosis. 心脏肉样瘤病管理指南的比较与对比。
Pub Date : 2020-01-01 Epub Date: 2020-08-31 DOI: 10.17996/anc.20-00123
David H Birnie, Niko Tzemos, Pablo B Nery

Introduction: The Japanese Circulation Society (JCS) recently published new guidelines for the diagnosis and treatment of Cardiac Sarcoidosis (CS). There are two other guideline documents, the World Association of Sarcoidosis and Other Granulomatous Disorders Sarcoidosis Organ (WASOG) Assessment Instrument created in 1999 and updated in 2014. Also, in 2014, the Heart Rhythm Society (HRS) published their international guideline document. As co-chair of the HRS document I have been invited to compare and contrast the management aspects of the HRS guidelines with the new JCS document. Comments: (i) The HRS document recommended a stepwise approach to VT management and the JCS document is somewhat similar; but with some key differences. (ii) The HRS statement suggested that an ICD for CS patients with an indication for a pacemaker "can be useful". The JCS document take a similar position although with some additional criteria related to National Health Institute Coverage guidelines. (iii) Both HRS and the JCS documents agree that ICDs are recommended in patients with general guideline indications for primary prevention (i.e. LVEF less than 35%). However which additional patients should be considered for ICDs is controversial. The 2016 JCS document is broadly similar, with the major exception that it is recommended that all patients with LVEF 35-50% should have an EP study. Conclusion: The Japanese have been leaders in many aspects of CS including in guideline development. It is clear that the future of CS management is bright, with increasing international collaborations and also multiple efforts underway to obtain higher quality data to inform future guidelines.

导言:日本循环学会(JCS)最近发布了新的心脏肉样瘤病(CS)诊断和治疗指南。另外还有两份指南文件,即世界肉样瘤病和其他肉芽肿性疾病协会肉样瘤病器官(WASOG)评估工具,该工具于 1999 年制定,并于 2014 年更新。此外,2014 年,心脏节律学会(HRS)发布了他们的国际指南文件。作为心律失常协会文件的联合主席,我受邀将心律失常协会指南的管理方面与新的 JCS 文件进行比较和对比。评论:(i) HRS 文件建议采用循序渐进的方法进行 VT 管理,JCS 文件与 HRS 文件有些类似,但也有一些关键的不同之处。(ii) HRS 声明建议,对于有起搏器适应症的 CS 患者,ICD "可能是有用的"。JCS 文件采取了类似的立场,但增加了一些与国家健康研究所覆盖指南相关的标准。(iii) HRS 和 JCS 文件都同意,对于具有一级预防一般指南指征的患者(即 LVEF 低于 35%),建议使用 ICD。然而,还有哪些患者应考虑使用 ICD 还存在争议。2016 年的 JCS 文件与之大体相似,主要的例外是建议所有 LVEF 为 35-50% 的患者都应进行 EP 研究。结论:日本人在 CS 的许多方面,包括指南制定方面一直处于领先地位。很明显,CS 管理的前景是光明的,国际合作不断增加,同时也在努力获取更高质量的数据,为未来的指南提供依据。
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引用次数: 0
Approach to Protecting Patients and Staff in Nuclear Cardiology Laboratories in Response to the COVID-19 Pandemic. 针对 COVID-19 大流行保护核心脏病学实验室患者和工作人员的方法。
Pub Date : 2020-01-01 Epub Date: 2020-08-31 DOI: 10.17996/anc.20-00133
Andrew Van Tosh, Kenneth J Nichols

The COVID-19 pandemic has altered all aspects of performing medical procedures throughout the world. It is important to stratify patients into categories according to the likelihood that a requested exam will result in a change in acute management. Health care staff should maintain adequate distancing and engage in frequent hand washing, and personnel who are patient-facing should put on PPE at all times. All patient-touching apparatus should be disinfected between patients according to the Infection Control protocols of the institutions. Most labs have chosen to have patients wear surgical masks to afford some level of protection for them. Efforts should be implemented to enable remote reading and remote reporting of study results. The guidelines presented in this paper are based on the currently available information regarding SARS-CoV-2 (COVID-19) viral infections, but it is essential that all laboratories comply with evolving recommendations of their institutions and public health authorities.

COVID-19 大流行改变了全世界医疗程序执行的方方面面。重要的是,要根据所要求的检查是否会导致急性病治疗的改变,将患者分层。医护人员应保持足够的距离并经常洗手,面对患者的人员应随时穿戴个人防护设备。所有接触患者的器械都应根据各机构的感染控制规程在患者之间进行消毒。大多数实验室都选择让患者佩戴外科口罩,为他们提供一定程度的保护。应努力实现远程读取和远程报告研究结果。本文中介绍的指导原则是基于目前可获得的有关 SARS-CoV-2 (COVID-19) 病毒感染的信息,但所有实验室都必须遵守其所在机构和公共卫生当局不断变化的建议。
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引用次数: 0
Paradoxical Prognostic Implications of Visceral Adiposity for Lethal Cardiac Events in Association with Impaired Cardiac Sympathetic Innervation in Chronic Heart Failure. 慢性心力衰竭中与心脏交感神经受损相关的致死性心脏事件内脏脂肪的矛盾预后意义。
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00118
Takahiro Doi, Tomoaki Nakata, Takahiro Noto, Tomohiro Mita, Satoshi Yuda, Akiyoshi Hashimoto

Background: Obesity increases the risk for development of heart failure (HF) but, when present is likely to be related to better outcomes in patients with HF. This study aimed to clarify the paradoxical prognostic values of visceral obesity in association with cardiac sympathetic function in HF patients. Methods and Results: A total of 653 consecutive patients with systolic HF who underwent visceral adiposity area (VAA) measurements using a computed tomographic technique were divided into 3 groups: VAA 1, area <80 cm2; VAA 2, area 80-140 cm2; VAA 3, area >140 cm2. Sympathetic innervation was quantified by 123I-MIBG cardiac activity. Patients were followed up for an average of 22 months with a primary endpoint of lethal cardiac events (CE). The CE group (n=200) had a lower late heart-to mediastinum ratio (HMR) and a smaller VAA than those in the non-CE group. Rates of overall CE/HF death were inversely correlated with VAA: 39.2% ± 6.2% for VAA 1, 27.4% ± 19.9% for VAA 2 and 24.1% ± 15.3% for VAA 3. In addition to sudden cardiac death rate, lethal arrhythmic event rate increased in association with visceral fat obesity: 3.0% for VAA 1, 7.5% for VAA 2 and 8.8% for VAA 3. Late HMR identified high-risk sub-populations in each group. Conclusion: Visceral obesity has paradoxical prognostic implications in terms of HF mortality and lethal arrhythmic/sudden cardiac death events. Cardiac sympathetic denervation and quantitative visceral adiposity are synergistically associated with overall cardiac mortality, contributing to better risk stratification of HF patients.

背景:肥胖增加心衰(HF)发生的风险,但肥胖可能与心衰患者更好的预后相关。本研究旨在阐明心衰患者内脏肥胖与心脏交感功能相关的矛盾的预后价值。方法与结果:653例连续收缩期心衰患者采用计算机断层扫描技术测量内脏脂肪区(VAA),分为3组:VAA 1,区2;VAA 2,面积80-140 cm2;VAA 3,面积>140 cm2。通过123I-MIBG心脏活动量化交感神经支配。患者平均随访22个月,主要终点为致死性心脏事件(CE)。与非CE组相比,CE组(n=200)晚期心脏与纵隔比值(HMR)和VAA均较低。CE/HF总死亡率与VAA呈负相关:VAA 1为39.2%±6.2%,VAA 2为27.4%±19.9%,VAA 3为24.1%±15.3%。除了心源性猝死外,致命心律失常事件发生率与内脏脂肪肥胖相关:VAA 1组为3.0%,VAA 2组为7.5%,VAA 3组为8.8%。晚期HMR确定了每组的高危亚人群。结论:内脏肥胖在心衰死亡率和致死性心律失常/心源性猝死事件方面具有矛盾的预后意义。心脏交感神经断动和定量内脏脂肪与心脏总死亡率协同相关,有助于更好地对心衰患者进行风险分层。
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引用次数: 2
ANC Opens up to the World 非洲国民大会向世界开放
Pub Date : 2020-01-01 DOI: 10.17996/anc.20-00001
K. Yoshinaga
A pproximately six years ago, the Japanese Society of Nuclear Cardiology (JSNC) launched its official journal, the Annals of Nuclear Cardiology (ANC), with the ultimate goal of advancing research and clinical practice by those in the field of nuclear cardiology, including physicians, technologists, and scientists, as well as the industries that support them. Initially JSNC aimed to promote its own members’ research and clinical practice and therefore it required any non-JSNC members who wanted to submit their manuscript to ANC to become JSNC members. However, as ANC has become bettter known among physicians and researchers on different continents and as the nature of research itself has moved further towards examining issues on a global scale, the time has come to open ANC not only to our members but to all nuclear cardiology researchers around the world. Beginning this year, ANC therefore no longer requires authors to become JSNC members or to pay a submission fee. With this change, ANC is now more accessible to everyone in the nuclear cardiology field around the world. In this spirit, the editorial board of ANC is working to ensure that articles published in ANC can be accessed via major search engines. Board members have been working very hard to collect the information necessary to apply to such search engines, but further steps are necessary. First, ANC needs to have a more international perspective. Therefore, the ANC editorial board is looking to include international advisory members among its ranks. This move will be coming soon. We are planning to apply to PubMed or PubMed Central operated by the National Institute of Health in the United States. Improving the quality of original articles and technical articles is now even more important and so last year we started an editorial support program. Through this program, editorial board members work with authors preparing manuscripts for submission. Beginning with this edition of ANC, original articles accepted for publication will have undergone thorough scientific editing as part of ANC’s process to support authors in manuscript preparation and to improve the overall quality of manuscripts. Authors sometimes overlook the new insight their studies may provide. In this regard, editorial board members, who have great depth of experience, will endeavour to help these authors to identify such new insights from their work. For this issue of ANC at least, the number of original articles is still limited. However, I expect this number to grow in the coming years. Along with increasing the quantity of manuscripts, we continuously aim to improve their quality. In this regard, the review process is very important. Therefore, I have asked experienced reviewers in Asia, the UK, Europe, North America, and South America to review articles submitted to ANC. Among ANC reviewers this year are a number of core American Society of Nuclear Cardiology members. I am especially grateful to those who have been wil
大约六年前,日本核心脏病学会(JSNC)推出了官方期刊《核心脏病学年鉴》(ANC),其最终目标是促进核心脏病学领域的研究和临床实践,包括医生、技术专家、科学家以及支持他们的行业。最初,JSNC旨在促进其成员的研究和临床实践,因此它要求任何想向ANC提交手稿的非JSNC成员成为JSNC成员。然而,随着ANC在不同大陆的医生和研究人员中越来越出名,随着研究本身的性质进一步向全球范围内的研究问题发展,现在不仅是向我们的成员开放ANC,而且是向全世界所有核心脏病学研究人员开放ANC的时候了。因此,从今年开始,ANC不再要求作者成为JSNC成员或支付提交费。随着这一变化,ANC现在对世界各地的核心脏病学领域的每个人都更容易获得。本着这种精神,ANC编辑委员会正在努力确保在ANC发表的文章可以通过主要搜索引擎访问。董事会成员一直在努力收集应用于此类搜索引擎所需的信息,但还需要采取进一步措施。首先,非国大需要有更多的国际视野。因此,非国大编辑委员会正在寻求将国际顾问成员纳入其行列。这一举措将很快出台。我们计划申请美国国立卫生研究院运营的PubMed或PubMed Central。提高原创文章和技术文章的质量现在变得更加重要,因此去年我们启动了一项编辑支持计划。通过这个项目,编辑委员会成员与作者一起准备投稿。从这一版ANC开始,接受发表的原创文章将经过彻底的科学编辑,作为ANC过程的一部分,以支持作者准备手稿并提高手稿的整体质量。作者有时会忽略他们的研究可能提供的新见解。在这方面,经验丰富的编辑委员会成员将努力帮助这些作者从他们的工作中发现这些新的见解。至少对于这一期的ANC来说,原创文章的数量仍然有限。然而,我预计这个数字在未来几年还会增长。随着稿件数量的增加,我们将不断提高稿件的质量。在这方面,审查进程非常重要。因此,我邀请了亚洲、英国、欧洲、北美和南美经验丰富的审稿人来审查提交给ANC的文章。在今年的ANC审稿人中,有一些是美国核心脏病学会的核心成员。我特别感谢那些在今年世界各地非常具有挑战性的情况下愿意抽出时间进行这些审查的人。我鼓励国际审稿人和研究人员考虑以审稿人或作者的身份参与ANC。为了向世界宣布其存在,ANC于2017年推出了其网站。从下图可以看出,今年对该网站的兴趣大大增加了。我希望,随着我们的期刊在全球核心脏病学界变得更加知名,来自世界各地的投稿数量也将继续增长。通过这种方式,随着原创文章质量和数量的不断提高,本刊将成为核心脏病学领域一个值得注意的贡献者。
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引用次数: 1
Can Visceral Adiposity Predict Paradoxical Prognostic Implications on Lethal Arrhythmic Events in Chronic Heart Failure Patients with Impaired Cardiac Sympathetic Activity? 内脏脂肪含量能否预测心脏交感神经活动受损的慢性心力衰竭患者致命性心律失常事件的反常预后影响?
Pub Date : 2020-01-01 Epub Date: 2020-08-31 DOI: 10.17996/anc.20-00126
Shu Kasama, Sang-Geon Cho
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引用次数: 0
The Diagnostic Criteria 2020 for Triglyceride Deposit Cardiomyovasculopathy. 甘油三酯沉积性心肌病诊断标准 2020》。
Pub Date : 2020-01-01 Epub Date: 2020-08-31 DOI: 10.17996/anc.20-00131
Kunihisa Kobayashi, Yasuhiko Sakata, Hideyuki Miyauchi, Yoshihiko Ikeda, Yasuyuki Nagasawa, Kenichi Nakajima, Kazunori Shimada, Junji Kozawa, Hiroyuki Hao, Tetsuya Amano, Hiroshi Yoshida, Tohru Inaba, Chikako Hashimoto, Ken-Ichi Hirano

Triglyceride deposit cardiomyovasculopathy (TGCV) is a newly identified disease that was discovered in individuals who required cardiac transplantation in Japan in 2008. Defective intracellular lipolysis causes triglyceride (TG) accumulation in the myocardium and coronary artery vascular smooth muscle cells, which results in severe heart failure and coronary artery disease with poor prognosis. A known cause of TGCV is a genetic deficiency of adipose triglyceride lipase (ATGL), a rate-limiting enzyme in the intracellular hydrolysis of TG. TGCV is classified into primary TGCV with ATGL mutations and idiopathic TGCV without ATGL mutations. Since its discovery, the Japan TGCV Study Group has attempted to elucidate its pathophysiology, develop diagnostic procedures, and specific treatment. Myocardial scintigraphy with iodine-123-β-methyl iodophenyl-pentadecanoic acid (123I-BMIPP) is a unique imaging modality for evaluating myocardial lipolysis in vivo. The washout rate of 123I-BMIPP is an essential indicator for the diagnosis of TGCV. Along with our efforts to provide awareness of and insights into this disease concept, we found that the cumulative number of clinically diagnosed patients has reached >200 and the cases are distributed throughout Japan. In addition, we successfully completed three investigator-initiated clinical trials of a potential therapeutic agent (CNT-01) for TGCV, which was assigned by the Ministry of Health, Labour, and Welfare, Japan, under the SAKIGAKE Designation System in June 2020. Here, we provide the Diagnostic Criteria 2020 for TGCV in order to further promote this "rare and intractable disease" project.

甘油三酯沉积性心肌病(TGCV)是一种新发现的疾病,2008 年在日本需要进行心脏移植的患者中被发现。细胞内脂肪分解缺陷会导致甘油三酯(TG)在心肌和冠状动脉血管平滑肌细胞内积聚,从而导致严重的心力衰竭和冠状动脉疾病,且预后不良。TGCV 的一个已知病因是脂肪甘油三酯脂肪酶(ATGL)的遗传缺陷,ATGL 是细胞内水解 TG 的限速酶。TGCV 可分为 ATGL 基因突变的原发性 TGCV 和无 ATGL 基因突变的特发性 TGCV。自发现以来,日本 TGCV 研究小组一直试图阐明其病理生理学,开发诊断程序和特定治疗方法。使用碘-123-β-甲基碘苯基十五烷酸(123I-BMIPP)进行心肌闪烁成像是一种独特的成像模式,可用于评估体内心肌脂肪溶解情况。123I-BMIPP 的洗脱率是诊断 TGCV 的重要指标。随着我们对这一疾病概念的认识和深入了解,我们发现临床诊断患者的累计人数已超过 200 人,病例分布在日本全国各地。此外,我们还成功完成了三项由研究者发起的 TGCV 潜在治疗药物(CNT-01)的临床试验,并于 2020 年 6 月获得日本厚生劳动省根据 "榊岳指定系统 "指定的治疗药物。在此,我们提供了 TGCV 的 2020 诊断标准,以进一步推动这一 "罕见难治疾病 "项目。
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引用次数: 0
期刊
Annals of nuclear cardiology
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