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Prospective Multicenter Screening With High-Sensitivity Cardiac Troponin T for Wild-Type Transthyretin Cardiac Amyloidosis in Outpatient and Community-Based Settings. 在门诊和社区环境中使用高敏心肌肌钙蛋白 T 对野生型转甲状腺素心脏淀粉样变性进行前瞻性多中心筛查。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-10-04 DOI: 10.1253/circj.CJ-24-0479
Naoki Arima, Yuri Ochi, Toru Kubo, Yoshinori Murakami, Kiyoshi Nishino, Hironori Yamamoto, Koji Satou, Shinjiro Tamura, Makoto Okawa, Hiroshi Takata, Yuji Shimizu, Yuichi Baba, Naohito Yamasaki, Hiroaki Kitaoka

Background: High-sensitivity cardiac troponin T (hs-cTnT) was proposed as a simple and useful diagnostic tool for cardiac amyloidosis (CA). We performed exploratory systemic screening using hs-cTnT to detect wild-type transthyretin CA (ATTRwt-CA) in outpatient and community-based settings.

Methods and results: This study was a prospective multicenter study including 8 internal medicine clinics in Kochi Prefecture, Japan. Consecutive individuals aged ≥70 years who visited those clinics as outpatients were enrolled. Patients with a prior diagnosis of CA or a history of heart failure hospitalization were excluded. We measured hs-cTnT levels in the enrolled individuals at each clinic, and those with elevated hs-cTnT levels (≥0.03ng/mL) received further detailed examination, including remeasurement of hs-cTnT. The diagnosis of ATTRwt-CA was confirmed by biopsy-proven transthyretin. Of 1,141 individuals enrolled in the study, 55 (4.8%) had elevated hs-cTnT levels. Of the 33 patients who underwent further examination, 22 had elevated hs-cTnT levels at remeasurement. Finally, 2 men were diagnosed with ATTRwt-CA. The prevalence of ATTRwt-CA was 9.1% (2/22) among patients with elevated hs-cTnT levels at two examinations, and at least 0.18% (2/1,141) in the whole study population.

Conclusions: Measurement of hs-cTnT will help to screen for patients with undiagnosed ATTRwt-CA in primary care practice.

背景:高敏心肌肌钙蛋白 T(hs-cTnT)被认为是诊断心脏淀粉样变性(CA)的简单而有用的工具。我们使用 hs-cTnT 对门诊和社区环境中的野生型转甲状腺素 CA(ATTRwt-CA)进行了探索性的系统筛查:本研究是一项前瞻性多中心研究,包括日本高知县的 8 家内科诊所。年龄≥70 岁的门诊患者连续入组。既往诊断为 CA 或有心力衰竭住院史的患者被排除在外。我们在每个诊所测量了入组患者的 hs-cTnT 水平,并对 hs-cTnT 水平升高(≥0.03ng/mL)的患者进行了进一步的详细检查,包括重新测量 hs-cTnT。ATTRwt-CA 的诊断由活检证实的转甲状腺素确诊。在 1141 名参与研究的患者中,55 人(4.8%)的 hs-cTnT 水平升高。在接受进一步检查的 33 名患者中,有 22 人在再次测量时发现 hs-cTnT 水平升高。最后,2 名男性患者被确诊为 ATTRwt-CA。在两次检查均出现 hs-cTnT 水平升高的患者中,ATTRwt-CA 的发病率为 9.1%(2/22),而在整个研究人群中,ATTRwt-CA 的发病率至少为 0.18%(2/1,141):结论:测量 hs-cTnT 将有助于在初级医疗实践中筛查未确诊的 ATTRwt-CA 患者。
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引用次数: 0
Prognostic Survey of ECPELLA in Japanese Patients With Acute Myocardial Infarction and Cardiogenic Shock - Findings From the Japanese Registry for Percutaneous Ventricular Assist Device (J-PVAD). 日本急性心肌梗死和心源性休克患者的 ECPELLA 预后调查--来自日本经皮心室辅助装置登记处 (J-PVAD) 的研究结果。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-10-02 DOI: 10.1253/circj.CJ-24-0522
Riku Arai, Nobuhiro Murata, Yuki Saito, Keisuke Kojima, Daisuke Fukamachi, Yasuo Okumura

Background: The short-term mortality associated with veno-arterial extracorporeal membrane oxygenation combined with the Impella device (termed ECPELLA) for acute myocardial infarction complicated by cardiogenic shock (AMI-CS) remains unclear.

Methods and results: The Japanese Registry for Percutaneous Ventricular Assist Devices (J-PVAD) includes data on all patients treated with an Impella in Japan. We extracted data for 922 AMI-CS patients who underwent ECPELLA support and conducted an exploratory analysis focusing on 30-day mortality. The median age of patients was 69 years, and 83.8% were male. The overall 30-day mortality was 46.1%. Factors associated with mortality included age >80 years, in-hospital cardiac arrest, systolic blood pressure <90 mmHg, serum creatinine >1.5 mg/dL, and serum lactate >4.0 mmol/L. In patients aged >80 years with any of these factors, mortality was significantly higher than in those without, ranging from 57.5% to 64.9%. The J-PVAD score assigns 1 point per predictor, with a C-statistic of 0.620 (95% confidence interval 0.586-0.654). The 30-day mortality was 20.0% for a J-PVAD score of 0, increasing to 70.0% for a score of 5.

Conclusions: The J-PVAD data indicate high short-term mortality in AMI-CS patients treated with ECPELLA, particularly among older patients. Further studies are needed to validate this risk stratification in this patient subset.

背景:静脉-动脉体外膜肺氧合联合 Impella 装置(称为 ECPELLA)治疗急性心肌梗死并发心源性休克(AMI-CS)的短期死亡率仍不清楚:日本经皮心室辅助装置登记处(J-PVAD)收录了日本所有使用 Impella 治疗患者的数据。我们提取了 922 名接受 ECPELLA 支持的 AMI-CS 患者的数据,并对 30 天死亡率进行了探索性分析。患者的中位年龄为 69 岁,83.8% 为男性。30 天内的总死亡率为 46.1%。与死亡率相关的因素包括年龄大于 80 岁、院内心脏骤停、收缩压 1.5 mg/dL、血清乳酸大于 4.0 mmol/L。年龄大于 80 岁且存在上述任一因素的患者的死亡率明显高于不存在上述任一因素的患者,从 57.5% 到 64.9% 不等。J-PVAD评分为每个预测因子打1分,C统计量为0.620(95%置信区间为0.586-0.654)。J-PVAD 评分为 0 时,30 天死亡率为 20.0%,评分为 5.结论时,死亡率上升至 70.0%:J-PVAD数据表明,接受ECPELLA治疗的AMI-CS患者短期死亡率较高,尤其是老年患者。需要进一步研究来验证这一患者亚群的风险分层。
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引用次数: 0
Machine Learning-Based Clustering Using a 12-Lead Electrocardiogram in Patients With a Implantable Cardioverter Defibrillator to Identify Future Ventricular Arrhythmia. 基于机器学习的聚类技术,利用植入式心脏除颤器患者的 12 导联心电图识别未来的室性心律失常。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-10-01 DOI: 10.1253/circj.CJ-24-0269
Ryo Tateishi, Masato Shimizu, Makoto Suzuki, Eiko Sakai, Atsuya Shimizu, Hiroshi Shimada, Nobutaka Katoh, Mitsuhiro Nishizaki, Tetsuo Sasano

Background: Implantable cardioverter defibrillators (ICDs) reduce mortality associated with ventricular arrhythmia in high-risk patients with cardiovascular disease. Machine learning (ML) approaches are promising tools in arrhythmia research; however, their application in predicting ventricular arrhythmias in patients with ICDs remains unexplored. We aimed to predict and stratify ventricular arrhythmias requiring ICD therapy using 12-lead electrocardiograms (ECGs) in patients with an ICD.

Methods and results: This retrospective analysis included 200 adult patients who underwent ICD implantation at a single center. Patient demographics, clinical features, and 12-lead ECG data were collected. Unsupervised learning techniques, including K-means and hierarchical clustering, were used to stratify patients based on 12-lead ECG features. Dimensionality reduction methods were also used to optimize clustering accuracy. The silhouette coefficient was used to determine the optimal method and number of clusters. Of the 200 patients, 59 (29.5%) received appropriate therapy. The mean age of patients was 62.3 years, and 81.0% were male. The mean follow-up period was 2,953 days, with no significant intergroup differences. Hierarchical clustering into 3 clusters proved to be the most accurate (silhouette coefficient=0.585). Kaplan-Meier curves for these 3 clusters revealed significant differences (P=0.026).

Conclusions: We highlight the potential of ML-based clustering using 12-lead ECGs to help in the risk stratification of ventricular arrhythmia. Future research in a larger multicenter setting may provide further insights and refine ICD indications.

背景:植入式心律转复除颤器(ICD)可降低心血管疾病高危患者与室性心律失常相关的死亡率。机器学习(ML)方法是心律失常研究领域前景广阔的工具,但其在预测 ICD 患者室性心律失常方面的应用仍有待探索。我们的目的是利用 ICD 患者的 12 导联心电图(ECG)对需要 ICD 治疗的室性心律失常进行预测和分层:这项回顾性分析包括在一个中心接受 ICD 植入术的 200 名成年患者。收集了患者的人口统计学特征、临床特征和 12 导联心电图数据。根据 12 导联心电图特征,采用包括 K-means 和分层聚类在内的无监督学习技术对患者进行分层。此外,还采用了降维方法来优化聚类的准确性。剪影系数用于确定最佳方法和聚类数量。在 200 名患者中,59 人(29.5%)接受了适当的治疗。患者的平均年龄为 62.3 岁,81.0% 为男性。平均随访时间为 2953 天,组间差异不明显。事实证明,将患者分成 3 个群组的分层聚类方法最为准确(剪影系数=0.585)。这3个聚类的Kaplan-Meier曲线显示出显著差异(P=0.026):我们强调了使用 12 导联心电图进行基于 ML 的聚类在帮助室性心律失常风险分层方面的潜力。未来在更大范围的多中心环境中进行的研究可能会提供更多见解,并完善 ICD 适应症。
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引用次数: 0
Left Atrial Low-Voltage Zone Assessment and Voltage-Guided Ablation Outcome in Patients With Atrial Fibrillation-Induced Tachycardiomyopathy. 心房颤动诱发心动过速患者的左心房低电压区评估和电压引导消融结果
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-28 DOI: 10.1253/circj.CJ-24-0079
Halim Marzak, Justine Hammann, Kensuke Matsushita, Romain Ringele, Simon Fitouchi, François Severac, Thomas Cardi, Mohamad Kanso, Alexandre Schatz, Patrick Ohlmann, Olivier Morel, Laurence Jesel

Background: Persistent atrial fibrillation (AF) patients with tachycardia-induced cardiomyopathy (TIC) undergoing catheter ablation have similar or even better outcomes than patients without TIC. Data regarding atrial substrate remodeling are scarce in cases of TIC. We assessed regional distribution of left atrial (LA) bipolar voltage, the extent of low-voltage zones (LVZs), and outcomes of voltage-guided ablation in AF patients with and without TIC.

Methods and results: In all, 139 patients with persistent AF presenting for a first voltage-guided catheter ablation were enrolled, 61 with TIC and 78 with structurally normal hearts. LA voltage maps were obtained using a 3-dimensional electroanatomical mapping system in sinus rhythm. LVZ was defined as <0.5 mV. Compared with non-TIC patients, TIC patients had a lower indexed LA volume (median [interquartile range] 58.6 [50.6-68.7] vs. 63.4 [60.1-76.1] mL/m2; P<0.01) and higher LA voltage (2.3 [1.5-2.8] vs. 1.7 [1-2.6] mV; P=0.02). LVZs were less frequently found in patients with than without TIC (8 [13.1%] vs. 30 [39%]; P<0.01). There was no significant difference in atrial tachyarrhythmia (AT)-free survival rate over a 36-month follow-up between the 2 groups (log-rank test, P=0.176). No predictor of AT recurrence was identified.

Conclusions: TIC patients exhibit less LA substrate remodeling with a smaller LA volume, higher bipolar voltage, and fewer LVZs than non-TIC patients. They have a similar favorable outcome after a single procedure.

背景:伴有心动过速诱发心肌病(TIC)的持续性心房颤动(AF)患者接受导管消融术的疗效与无 TIC 的患者相似,甚至更好。有关 TIC 病例中心房基底重塑的数据很少。我们评估了有TIC和无TIC的房颤患者左心房(LA)双极电压的区域分布、低电压区(LVZ)的范围以及电压引导消融的结果:共有 139 名首次接受电压引导导管消融术的持续性房颤患者,其中 61 人患有 TIC,78 人心脏结构正常。在窦性心律状态下,使用三维电解剖绘图系统获得 LA 电压图。LVZ定义为2;PC结论:与非 TIC 患者相比,TIC 患者的 LA 基底重塑较少,LA 容积较小,双极电压较高,LVZ 较少。与非 TIC 患者相比,TIC 患者的 LA 基底重塑较少,LA 容积较小,双极电压较高,LVZ 较少,单次手术后的良好预后相似。
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引用次数: 0
Right Ventricular to Pulmonary Artery Uncoupling Is Associated With Impaired Exercise Capacity in Patients With Transthyretin Cardiac Amyloidosis. 右心室与肺动脉解偶联与传导色素性心脏淀粉样变性患者运动能力受损有关。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-28 DOI: 10.1253/circj.CJ-24-0402
Toru Hashimoto, Kei Ikuta, Shoei Yamamoto, Tomoaki Yoshitake, Tomoyasu Suenaga, Shunsuke Nakashima, Takashi Kai, Kayo Misumi, Takeo Fujino, Keisuke Shinohara, Shouji Matsushima, Rina Atsumi, Takuro Isoda, Shintaro Kinugawa, Kohtaro Abe

Background: Exercise capacity is related to mortality and morbidity in heart failure (HF) patients. Determinants of exercise capacity in transthyretin cardiac amyloidosis (ATTR-CA) have not been established.

Methods and results: This single-center study retrospectively evaluated ATTR-CA patients and patients with non-amyloidosis HF with preserved/mildly reduced ejection fraction (HFpEF/HFmrEF) (n=32 and n=51, respectively). In the ATTR-CA group, the median age was 75.5 years (interquartile range [IQR] 71.3-78.8 years), 90.6% were male, and the median left ventricular (LV) ejection fraction was 53.5% (IQR 41.4-65.6%). Cardiopulmonary exercise tests revealed a median peak oxygen consumption and anaerobic threshold of 15.9 (IQR 11.6-17.4) and 10.6 (IQR 8.5-12.0] mL/min/kg, respectively, and ventilatory efficiency (minute ventilation/carbon dioxide production [V̇E/V̇CO2] slope) of 35.5 (IQR 32.0-42.5). Among exercise variables, V̇E/V̇CO2slope has the greatest prognostic value. Univariate analysis revealed a significant correlation between V̇E/V̇CO2slope and age, LV global longitudinal strain, tricuspid annular plain systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) ratio, and mixed venous oxygen saturation. In multivariate analyses, the TAPSE/PASP ratio was an independent predictor of V̇E/V̇CO2slope (95% confidence interval -44.5, -10.8; P=0.0067). In non-amyloidosis HFpEF/HFmrEF patients, the TAPSE/PASP ratio was not independently correlated with V̇E/V̇CO2slope.

Conclusions: Right ventricular-pulmonary artery coupling estimated by the TAPSE/PASP ratio determines exercise capacity in ATTR-CA patients. This highlights the importance of early therapeutic intervention against underappreciated right ventricular dysfunction associated with ATTR-CA.

背景:运动能力与心力衰竭(HF)患者的死亡率和发病率有关:运动能力与心力衰竭(HF)患者的死亡率和发病率有关。转甲状腺素心脏淀粉样变性(ATTR-CA)患者运动能力的决定因素尚未确定:这项单中心研究回顾性评估了 ATTR-CA 患者和射血分数保留/轻度降低(HFpEF/HFmrEF)的非淀粉样变性高血压患者(分别为 32 人和 51 人)。在ATTR-CA组中,中位年龄为75.5岁(四分位数间距[IQR] 71.3-78.8岁),90.6%为男性,中位左心室射血分数为53.5%(IQR 41.4-65.6%)。心肺运动测试显示,中位峰值耗氧量和无氧阈值分别为 15.9(IQR 11.6-17.4)和 10.6(IQR 8.5-12.0)毫升/分钟/千克,通气效率(分钟通气量/二氧化碳产生量[V.E./V.CO2]斜率)为 35.5(IQR 32.0-42.5)。在运动变量中,通气量/二氧化碳产生量斜率具有最大的预后价值。单变量分析显示,V鄄E/V鄄CO2slope与年龄、左心室整体纵向应变、三尖瓣环平原收缩期偏移/肺动脉收缩压(TAPSE/PASP)比值和混合静脉血氧饱和度之间存在显著相关性。在多变量分析中,TAPSE/PASP比值是V̇E/V̇CO2slope的独立预测因子(95%置信区间-44.5,-10.8;P=0.0067)。在非淀粉样变性HFpEF/HFmrEF患者中,TAPSE/PASP比值与V.J.E/V.J.CO2slope无独立相关性:通过TAPSE/PASP比值估测的右心室-肺动脉耦合决定了ATTR-CA患者的运动能力。这凸显了针对 ATTR-CA 相关右心室功能障碍进行早期治疗干预的重要性。
{"title":"Right Ventricular to Pulmonary Artery Uncoupling Is Associated With Impaired Exercise Capacity in Patients With Transthyretin Cardiac Amyloidosis.","authors":"Toru Hashimoto, Kei Ikuta, Shoei Yamamoto, Tomoaki Yoshitake, Tomoyasu Suenaga, Shunsuke Nakashima, Takashi Kai, Kayo Misumi, Takeo Fujino, Keisuke Shinohara, Shouji Matsushima, Rina Atsumi, Takuro Isoda, Shintaro Kinugawa, Kohtaro Abe","doi":"10.1253/circj.CJ-24-0402","DOIUrl":"https://doi.org/10.1253/circj.CJ-24-0402","url":null,"abstract":"<p><strong>Background: </strong>Exercise capacity is related to mortality and morbidity in heart failure (HF) patients. Determinants of exercise capacity in transthyretin cardiac amyloidosis (ATTR-CA) have not been established.</p><p><strong>Methods and results: </strong>This single-center study retrospectively evaluated ATTR-CA patients and patients with non-amyloidosis HF with preserved/mildly reduced ejection fraction (HFpEF/HFmrEF) (n=32 and n=51, respectively). In the ATTR-CA group, the median age was 75.5 years (interquartile range [IQR] 71.3-78.8 years), 90.6% were male, and the median left ventricular (LV) ejection fraction was 53.5% (IQR 41.4-65.6%). Cardiopulmonary exercise tests revealed a median peak oxygen consumption and anaerobic threshold of 15.9 (IQR 11.6-17.4) and 10.6 (IQR 8.5-12.0] mL/min/kg, respectively, and ventilatory efficiency (minute ventilation/carbon dioxide production [V̇<sub>E</sub>/V̇<sub>CO2</sub>] slope) of 35.5 (IQR 32.0-42.5). Among exercise variables, V̇<sub>E</sub>/V̇<sub>CO2</sub>slope has the greatest prognostic value. Univariate analysis revealed a significant correlation between V̇<sub>E</sub>/V̇<sub>CO2</sub>slope and age, LV global longitudinal strain, tricuspid annular plain systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) ratio, and mixed venous oxygen saturation. In multivariate analyses, the TAPSE/PASP ratio was an independent predictor of V̇<sub>E</sub>/V̇<sub>CO2</sub>slope (95% confidence interval -44.5, -10.8; P=0.0067). In non-amyloidosis HFpEF/HFmrEF patients, the TAPSE/PASP ratio was not independently correlated with V̇<sub>E</sub>/V̇<sub>CO2</sub>slope.</p><p><strong>Conclusions: </strong>Right ventricular-pulmonary artery coupling estimated by the TAPSE/PASP ratio determines exercise capacity in ATTR-CA patients. This highlights the importance of early therapeutic intervention against underappreciated right ventricular dysfunction associated with ATTR-CA.</p>","PeriodicalId":50691,"journal":{"name":"Circulation Journal","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142331700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Myocardial Infarction as the Initial Presentation of Cardiac Papillary Fibroelastoma - Detection of a Mottled Mass Using Intravascular Ultrasound. 心肌梗死是心脏乳头状纤维母细胞瘤的最初表现--利用血管内超声波检测斑块。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-25 Epub Date: 2024-08-08 DOI: 10.1253/circj.CJ-24-0529
Yuki Kobayashi, Hidehiro Iwakawa, Takayuki Yamanaka, Katsuhito Seki, Hiroyuki Watanabe
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引用次数: 0
Greetings From the New Editor-in-Chief. 新主编致辞
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-25 Epub Date: 2024-09-09 DOI: 10.1253/circj.CJ-66-0231
Kenichi Tsujita
{"title":"Greetings From the New Editor-in-Chief.","authors":"Kenichi Tsujita","doi":"10.1253/circj.CJ-66-0231","DOIUrl":"10.1253/circj.CJ-66-0231","url":null,"abstract":"","PeriodicalId":50691,"journal":{"name":"Circulation Journal","volume":" ","pages":"1603-1604"},"PeriodicalIF":3.1,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142156503","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease. 验证 2018 年 IgG4 相关性大动脉周围炎/动脉周围炎和腹膜后纤维化(IgG4PA/RPF)诊断标准,并提出 2023 年 IgG4 相关性心血管/腹膜后疾病修订版。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-25 Epub Date: 2024-03-16 DOI: 10.1253/circj.CJ-24-0026
Ichiro Mizushima, Noriyasu Morikage, Eisaku Ito, Fuminori Kasashima, Yasushi Matsumoto, Naoki Sawa, Hajime Yoshifuji, Takako Saeki, Yukako Shintani-Domoto, Shogo Shimada, Toshio Takayama, Eisuke Amiya, Makiko Ozawa, Masaaki Takahashi, Yasunari Fujinaga, Takahiro Katsumata, Yukio Obitsu, Atsushi Izawa, Hiroyuki Kanno, Noriko Oyama-Manabe, Nobukazu Ishizaka, Tasuku Nagasawa, Hiroki Takahashi, Takao Ohki, Mitsuhiro Kawano, Satomi Kasashima

Background: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.

Methods and results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients ("mimickers") identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts' diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating "radiologic findings of pericarditis", "eosinophilic infiltration or lymphoid follicles", and "probable diagnosis of extra-PA/-RPF lesions", improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively.

Conclusions: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

背景:2018年,IgG4相关性大动脉周围炎/动脉周围炎和腹膜后纤维化(PA/RPF)的诊断标准被引入。本研究对现有标准进行了评估,并制定了改进版本:2022年8月至2023年1月期间,我们回顾性分析了110例确诊为IgG4相关疾病(IgG4-RD)并伴有心血管和/或腹膜后表现的日本患者,以及由专家确定的73例非IgG4-RD患者("模仿者")。患者被分为推导组(88 人)和验证组(95 人)。IgG4-RD或非IgG4-RD的分类基于2018年诊断标准和各种修订版本。灵敏度和特异性以专家诊断作为诊断真正 IgG4-RD 和拟态的金标准进行计算。在推导组中,2018 年标准显示出 58.5% 的灵敏度和 100% 的特异性。修订版纳入了 "心包炎的放射学发现"、"嗜酸性粒细胞浸润或淋巴滤泡 "和 "PA/RPF 外病变的可能诊断",将敏感性提高到 69.8%,同时保持了 100%的特异性。在验证组中,原始标准和修订标准的敏感性分别为 68.4% 和 77.2%,特异性分别为 97.4% 和 94.7%:拟议的 2023 年修订版 IgG4 相关心血管/腹膜后疾病标准通过纳入放射学、病理学和心血管/腹膜外器官类别的新项目,在保留高特异性的同时显著提高了灵敏度。
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引用次数: 0
Necrotic Change of Tunica Media Plays a Key Role in the Development of Coronary Artery Lesions in Kawasaki Disease. 川崎病冠状动脉病变发生的关键因素是鳞状上皮的坏死性改变。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-25 Epub Date: 2024-08-21 DOI: 10.1253/circj.CJ-24-0295
Seigo Okada, Aiko Sakai, Yuji Ohnishi, Hiroki Yasudo, Takahiro Motonaga, Reiji Fukano, Takako Waniishi, Masaya Sugiyama, Shunji Hasegawa

Background: Alarmins resulting from cell death or oxidative stress are involved in the development of Kawasaki disease (KD) vasculitis. In a previous study, we demonstrated the potential role of interleukin (IL)-33 as an alarmin in the development of KD vasculitis. Although edematous dissociation (necrotic change) of the tunica media is thought to be a major source of IL-33 in KD vasculitis, it has not yet been elucidated.

Methods and results: We investigated the impact of IL-33 released from necrotic human coronary artery smooth muscle cells (HCASMCs) on human coronary artery endothelial cells (HCAECs) using a coculture assay. Subsequently, we evaluated the anti-inflammatory effects of anti-IL-33 and anti-suppression of tumorigenicity 2 (ST2) antibodies compared with conventional therapies of KD, such as high-dose IgG or anti-tumor necrosis factor (TNF)-α antibody. Primary necrosis of HCASMCs induced significant release of IL-33. In cocultures of necrotic HCASMCs with HCAECs, the necrotic HCASMCs significantly induced the production of various proinflammatory cytokines in the HCAECs. Anti-IL-33 and anti-ST2 antibodies exhibited unique inhibitory effects on the production of platelet-derived growth factor-BB or IL-12(p70) in HCAECs.

Conclusions: There is potential involvement of edematous dissociation of the tunica media in the development of KD vasculitis. Furthermore, the distinctive anti-inflammatory effects of the anti-IL-33/ST2 axis drugs suggest novel therapeutic options for patients with refractory KD.

背景:细胞死亡或氧化应激导致的警戒素参与了川崎病(KD)血管炎的发病。在之前的一项研究中,我们证实了白细胞介素(IL)-33作为一种警戒素在川崎病血管炎发病过程中的潜在作用。虽然中膜水肿性解离(坏死性改变)被认为是 KD 脉管炎中 IL-33 的主要来源,但尚未得到阐明:我们使用共培养试验研究了坏死的人冠状动脉平滑肌细胞(HCASMCs)释放的IL-33对人冠状动脉内皮细胞(HCAECs)的影响。随后,我们评估了抗IL-33和抗抑制肿瘤生成2(ST2)抗体与KD传统疗法(如大剂量IgG或抗肿瘤坏死因子(TNF)-α抗体)相比的抗炎效果。HCASMCs的原发性坏死会诱导IL-33的大量释放。在坏死的 HCASMCs 与 HCAECs 的共培养中,坏死的 HCASMCs 能显著诱导 HCAECs 产生各种促炎细胞因子。抗IL-33和抗ST2抗体对HCAECs中血小板衍生生长因子-BB或IL-12(p70)的产生有独特的抑制作用:结论:KD血管炎的发生可能与中膜水肿解离有关。此外,抗IL-33/ST2轴药物的独特抗炎作用为难治性KD患者提供了新的治疗选择。
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引用次数: 0
Do You Prescribe PCSK9 Inhibitors Properly? 您是否正确处方 PCSK9 抑制剂?
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-09-25 Epub Date: 2024-03-29 DOI: 10.1253/circj.CJ-24-0186
Masatsune Ogura
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引用次数: 0
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