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The dilemma of endomyocardial biopsy sampling for lymphocytic myocarditis diagnosis 淋巴细胞性心肌炎诊断中心肌内膜活检取样的困境。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-09-04 DOI: 10.1016/j.carpath.2025.107778
Tatsuya Shiraki, Rika Kawakami, Renu Virmani
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引用次数: 0
Circ_0005372 targets the miR-153-3p/ITGB3 axis to stimulate the PI3K/AKT signaling pathway to facilitate the occurrence and development of congenital heart disease and pulmonary arterial hypertension in children Circ_0005372靶向miR-153-3p/ITGB3轴,刺激PI3K/AKT信号通路,促进儿童先天性心脏病和肺动脉高压的发生发展。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-27 DOI: 10.1016/j.carpath.2025.107768
Mingyan Zhu , Guixiang Li , Yungui Li , Jinsong Chen

Background

Congenital heart disease (CHD) is the most common and highest incidence of congenital malformations in newborn infants. Pulmonary arterial hypertension (PAH) is the most serious complication associated with CHD. It is a great threat to the safety and health of newborns.

Methods

The expression of circ_0005372, miR-153-3p, and ITGB3 were detected by Real-time quantitative PCR (RT-qPCR) and western blot (WB). MTT and EdU assays were used to evaluate the viability and proliferation of human pulmonary artery smooth muscle cells (HPASMC). Migration and invasion abilities were determined by wound healing and Transwell assays. Pull down and RNA Immunoprecipitation (RIP), and dual luciferase reporter gene assays were used to detect targeting relationships of circ_0005372, miR-153-3p, and ITGB3.

Results

Plasma samples from 27 healthy, CHD, and PAHCHD pairs of newborns were collected. Circ_0005372 was highly expressed in PAHCHD patient samples and HPASMCs under hypoxic conditions. Knockdown of circ_0005372 inhibited HPASMCs viability, proliferation, migration, and invasion. Circ_0005372 targeted miR-153-3p and showed a negative correlation. The inhibitory effect of silencing circ_0005372 on the HPASMCs was reversed by miR-153-3p inhibitor. MiR-153-3p negatively regulated ITGB3, and overexpression of ITGB3 abolished the effect of miR-153-3p in hypoxia-treated HPASMCs. Circ_0005372 could regulate the PI3K/AKT signaling pathway through miR-153-3p/ ITGB3.

Conclusion

In summary, circ_0005372 mediates the expression of ITG3B via targeting miR-153-3p, thereby stimulating the PI3K/AKT signaling pathway, helping the proliferation, migration, and invasion of HPASMCs and accelerating the deterioration of PAHCHD.
背景:先天性心脏病(CHD)是新生儿中最常见、发病率最高的先天性畸形。肺动脉高压(PAH)是冠心病最严重的并发症。这是对新生儿安全和健康的巨大威胁。方法:采用实时荧光定量PCR (RT-qPCR)和western blot (WB)检测circ_0005372、miR-153-3p、ITGB3的表达。采用MTT和EdU测定人肺动脉平滑肌细胞(HPASMC)的活力和增殖能力。通过伤口愈合和Transwell试验测定迁移和侵袭能力。使用Pull - down和RNA免疫沉淀(RIP)以及双荧光素酶报告基因检测circ_0005372、miR-153-3p和ITGB3的靶向关系。结果:收集了27对健康、冠心病和PAH-CHD新生儿的血浆样本。Circ_0005372在缺氧条件下的PAH-CHD患者样本和HPASMCs中高表达。敲低circ_0005372抑制HPASMCs的活力、增殖、迁移和侵袭。Circ_0005372靶向miR-153-3p,呈负相关。沉默circ_0005372对HPASMCs的抑制作用被miR-153-3p抑制剂逆转。MiR-153-3p负性调节ITGB3, ITGB3过表达可消除MiR-153-3p在缺氧处理的HPASMCs中的作用。Circ_0005372可通过miR-153-3p/ ITGB3调控PI3K/AKT信号通路。结论:综上所述,circ_0005372通过靶向miR-153-3p介导ITG3B的表达,从而刺激PI3K/AKT信号通路,促进hpasmc的增殖、迁移和侵袭,加速PAH-CHD的恶化。
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引用次数: 0
Anatomical variations of the mitral leaflets: Unified and accessory scallops 二尖瓣小叶的解剖变异:统一扇贝和附属扇贝
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-23 DOI: 10.1016/j.carpath.2025.107767
Buse Naz ÇANDIR GÜRSES , Kader YILAR , Çağla ERGİN , Özcan GAYRETLİ

Aim

To investigate the morphological variants of the leaflets and scallops of the mitral valve in fresh hearts of healthy individuals and to determine their morphometric values.

Methods

The hearts of 100 autopsy cases were analysed. The weight and dimensions of the heart and mitral valve circumference were measured. The morphology of the mitral leaflets and scallops and their hinge distances and heights were recorded.

Results

In 64 cases, classical leaflet morphology (aortic leaflet, mural leaflet consisting of 3 scallops, superolateral and inferoseptal commissures) was observed. In 24 cases accessory scallops and in 12 cases unified scallops were identified. The mean hinge distance and height of accessory scallops were 8.69±2.5 mm, and 9.79±2.5 mm respectively, and these values were 45.73±9.8 mm, and 11.95±2.3 mm in unified scallops, respectively. Accessory and unified scallops can be found in any region of mural leaflet, but the vast majority (96 %) are associated with it and more than half (60 %) are located at the commissural ends. In 1 case aortic leaflet consisted of two scallops and mural leaflet had 3 scallops in 63 %, 4 scallops in 22 %, 2 scallops in 8 %, 1 scallop in 4 % and 5 scallops in 3 %.

Conclusion

Significant morphological variations between mitral leaflets and scallops have been reported. These variations may affect the placement of the suture or device in mitral valve repair. It was also concluded that these variations cannot be predicted by age, gender or cardiac morphometry.
目的研究健康人新鲜心脏二尖瓣小叶和扇贝的形态变异,并测定其形态学值。方法对100例尸体解剖的心脏进行分析。测量心脏重量、尺寸及二尖瓣周长。记录二尖瓣小叶和扇贝的形态及其铰链的距离和高度。结果64例观察到典型的小叶形态(主动脉小叶、由3个扇贝组成的壁小叶、上外侧小叶和隔间小叶)。其中,辅助扇贝24例,统一扇贝12例。附属扇贝的平均铰链距离和高度分别为8.69±2.5 mm和9.79±2.5 mm,统一扇贝的平均铰链距离和高度分别为45.73±9.8 mm和11.95±2.3 mm。附扇贝和统一扇贝可以在壁画小叶的任何区域发现,但绝大多数(96%)与之相关,超过一半(60%)位于联合端。1例主动脉瓣叶由2个扇贝组成,壁瓣叶有3个扇贝占63%,4个扇贝占22%,2个扇贝占8%,1个扇贝占4%,5个扇贝占3%。结论二尖瓣小叶与扇贝有明显的形态学差异。这些变化可能会影响缝线或二尖瓣修复装置的位置。我们还得出结论,这些变化不能通过年龄、性别或心脏形态测定来预测。
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引用次数: 0
Fatal rupture of a giant left coronary artery aneurysm in an infant with Kawasaki disease. A case report with systematic literature review 一名患有川崎病的婴儿巨大左冠状动脉瘤破裂致死性。一例报告并系统文献复习
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-18 DOI: 10.1016/j.carpath.2025.107766
Jonathan K Lai , José Luis López-Guillén , Rae SM Yeung , Mamoru Ayusawa , Brian W McCrindle , Anita Nagy
Kawasaki disease (KD) is a systemic vasculitis of childhood that may lead to coronary artery aneurysms. Rupture of a giant coronary aneurysm is an exceptionally rare but often fatal complication. We report the case of a 4-month-old infant with treatment-resistant Kawasaki disease who developed rapidly enlarging giant aneurysms in all coronary artery branches despite aggressive immunomodulatory therapy. The patient died from cardiac tamponade due to rupture of a giant left anterior descending coronary artery aneurysm. Full autopsy revealed widespread vasculitis affecting multiple visceral arteries in addition to coronary artery involvement. Histological analysis demonstrated panarteritis with medial destruction and no evidence of thrombosis or myocardial infarction. A systematic review of reported cases demonstrated comparable clinical patterns and outcomes. This report underscores the potential for systemic vascular involvement in severe Kawasaki disease and highlights the importance of early recognition and intensified treatment in refractory cases.
川崎病是一种儿童期全身性血管炎,可导致冠状动脉瘤。巨大冠状动脉瘤破裂是一种非常罕见但往往致命的并发症。我们报告一例4个月大的婴儿患有治疗抵抗性川崎病,尽管积极的免疫调节治疗,但在所有冠状动脉分支中发展迅速扩大的巨大动脉瘤。病人死于巨大的左冠状动脉前降动脉瘤破裂引起的心包填塞。全面尸检显示广泛的血管炎,除了冠状动脉受累外,还影响到多个内脏动脉。组织学分析显示泛动脉炎伴内侧破坏,无血栓形成或心肌梗死的证据。对报告病例的系统回顾显示了可比较的临床模式和结果。本报告强调了严重川崎病累及全身血管的可能性,并强调了对难治性病例早期识别和强化治疗的重要性。
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引用次数: 0
Revising the histopathologic definition of myocarditis: Birth of the seaport criteria 修订心肌炎的组织病理学定义:海港标准的诞生。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-15 DOI: 10.1016/j.carpath.2025.107765
Gregory A. Fishbein , Stephen D. Preston
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引用次数: 0
Immuhistochemically-confirmed mitochondrial cardiomyopathy presenting as a conduction system hamartoma: A case report 免疫组织化学证实线粒体心肌病表现为传导系统错构瘤:1例报告。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-13 DOI: 10.1016/j.carpath.2025.107764
Ryo Kaimori , Kentaro Sakai , Atsuhito Takeda , Rina Hayata , Shinji Yano , Haruto Nishida , Tsutomu Daa , Shinjiro Mori
Conduction system hamartoma (CSH) is a rare cardiac lesion characterized by the abnormal proliferation of Purkinje-like myocytes. It predominantly affects female infants and is often associated with sudden cardiac death. Recent studies have linked mitochondrial dysfunction, particularly complex I deficiency, with CSH. We report an autopsy case of an eight-month-old female infant who died suddenly following mild gastrointestinal symptoms. A gross examination revealed mild cardiac hypertrophy without nodular lesions. Histological analysis identified multifocal aggregates of Purkinje-like cells with clear or foamy cytoplasm, some forming well-circumscribed nodules in the non-compacted myocardium. Immunohistochemistry demonstrated a marked reduction in complex I expression, supporting mitochondrial dysfunction. Although prominent trabeculations and deep recesses suggestive of left ventricular noncompaction were observed, they did not meet the strict diagnostic criteria. This case supports the potential role of mitochondrial complex I deficiency as a key pathogenic mechanism in CSH and highlights the significance of detailed histopathological and immunohistochemical analyses for an accurate diagnosis, especially in cases of sudden unexplained infant death.
传导系统错构瘤(CSH)是一种罕见的心脏病变,其特征是浦金氏样肌细胞异常增殖。它主要影响女婴,并常与心源性猝死有关。最近的研究将线粒体功能障碍,特别是复合物I缺乏与CSH联系起来。我们报告一个尸检病例八个月大的女婴谁突然死亡后轻微的胃肠道症状。大体检查显示心脏轻度肥大,无结节性病变。组织学分析发现浦肯病样细胞多灶聚集,胞浆呈透明或泡沫状,在未致密的心肌中形成界限清晰的结节。免疫组织化学显示复合物I表达明显减少,支持线粒体功能障碍。虽然观察到明显的小梁和深凹提示左心室不压实,但它们不符合严格的诊断标准。该病例支持了线粒体复合物I缺乏作为CSH关键致病机制的潜在作用,并强调了详细的组织病理学和免疫组织化学分析对于准确诊断的重要性,特别是在婴儿猝死原因不明的情况下。
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引用次数: 0
COVER 3: Editorial Board 封面3:编辑委员会
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-05 DOI: 10.1016/S1054-8807(25)00040-7
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引用次数: 0
COVER 4: Table of Contents/Barcode PMS 200 封面4:目录/条形码PMS 200
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-08-05 DOI: 10.1016/S1054-8807(25)00041-9
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引用次数: 0
A case of cardiac undifferentiated pleomorphic sarcoma treated with post-operative radiotherapy followed by heart transplantation 心脏未分化多形性肉瘤术后放疗合并心脏移植1例。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-07-29 DOI: 10.1016/j.carpath.2025.107760
Sungyeon Jung , Eun Na Kim , Hye In Lee , Hak Jae Kim , Jiwon Koh
Cardiac undifferentiated pleomorphic sarcomas (UPS) are extremely rare tumors that typically arise in the left atrium. They behave highly aggressively, requiring multimodality treatment when complete surgical resection is not feasible. Nevertheless, there is ongoing debate regarding the efficacy of adjuvant radiation therapy and the associated risk of radiation-induced cardiotoxicity. Pathologically, there is an emerging connection between cardiac UPS and intimal sarcoma of the great vessels, supported by morphological and genetic similarities. Here, we present the case of a 63-year-old woman diagnosed with a large right ventricular UPS. The patient underwent incomplete tumor resection, which was followed by post-operative radiotherapy. After nine months, her cardiac function deteriorated rapidly, and she ultimately ended up requiring heart transplantation. We discuss this case in the context of the existing literature on the diagnosis and treatment of cardiac UPS, focusing on the histopathologic features of the heart after radiotherapy.
心脏未分化多形性肉瘤(UPS)是一种罕见的肿瘤,通常发生在左心房。它们具有很强的侵袭性,当完全手术切除不可行时,需要多模式治疗。然而,关于辅助放射治疗的疗效和放射引起的心脏毒性的相关风险仍存在争议。在病理学上,在形态学和遗传相似性的支持下,心脏UPS和大血管内膜肉瘤之间有新的联系。在这里,我们提出的情况下,63岁的妇女诊断为一个大的右心室UPS。患者行肿瘤不完全切除,术后行放疗。9个月后,她的心脏功能迅速恶化,最终需要心脏移植。我们结合已有的关于心脏UPS的诊断和治疗的文献,重点讨论放疗后心脏的组织病理学特征。
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引用次数: 0
Lymphocytic myocarditis: A histopathologic definition and classification from the Society for Cardiovascular Pathology and Association for European Cardiovascular Pathology. I: Endomyocardial biopsy 淋巴细胞性心肌炎:来自心血管病理学会和欧洲心血管病理协会的组织病理学定义和分类。I:心内膜肌活检。
IF 1.9 4区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-07-23 DOI: 10.1016/j.carpath.2025.107759
Marc K. Halushka , Giulia d’Amati , Melanie C. Bois , John T. Fallon , Carla Giordano , Karin Klingel , Charles Leduc , Richard N. Mitchell , Stefania Rizzo , Cristina Basso

Background and aim

Lymphocytic myocarditis has long been appreciated as a lymphocyte-predominant myocardial inflammation with resultant myocyte injury. However, current methods of diagnosis on endomyocardial biopsy (EMB) lead to inconsistent diagnoses. To improve patient care, the criteria for the diagnosis of lymphocytic myocarditis on endomyocardial biopsies have been revised to address shortcomings of the Dallas Criteria and European Society of Cardiology (ESC) criteria.

Methods and results

In the Seaport area of Boston, a panel of expert cardiovascular pathologists from the Society for Cardiovascular Pathology (SCVP) and the Association for European Cardiovascular Pathology (AECVP) completed a three-year project to develop consensus terms and definitions. These “Seaport” criteria for EMB were developed to utilize CD3 immunohistochemistry for T lymphocytes and accurately define each level of histopathologic severity based on extent of infiltrate and presence of myocyte injury. These criteria create a diagnostic approach to lymphocytic myocarditis using three grades of severity (mild, moderate, and severe) along with a category of scattered increased T lymphocytes of undetermined significance (SITUS). The document discusses the role of ancillary studies, the relationship to other diagnostic modalities, and areas of continuing controversy in the development of these criteria.

Conclusion

These consensus-based criteria offer a standardized framework for diagnosing lymphocytic myocarditis in EMB. Adoption of these easy-to-use criteria will improve classification of lymphocytic myocarditis as an aid to diagnose and treat patients.
背景和目的:淋巴细胞性心肌炎长期以来被认为是一种以淋巴细胞为主的心肌炎症,导致心肌细胞损伤。然而,目前的诊断方法的心内膜肌活检(EMB)导致不一致的诊断。为了改善患者的护理,为了解决达拉斯标准和欧洲心脏病学会(ESC)标准的不足,对心内膜肌活检诊断淋巴细胞性心肌炎的标准进行了修订。方法和结果:在波士顿的海港地区,来自心血管病理学学会(SCVP)和欧洲心血管病理学协会(AECVP)的心血管病理学专家小组完成了一项为期三年的项目,以制定共识的术语和定义。这些EMB的“海港”标准是利用T淋巴细胞的CD3免疫组织化学,并根据浸润程度和肌细胞损伤的存在准确地定义每个组织病理严重程度。这些标准创建了一种淋巴细胞性心肌炎的诊断方法,使用三个严重等级(轻度、中度和重度)以及一类不确定意义的分散增加的T淋巴细胞(SITUS)。该文件讨论了辅助研究的作用,与其他诊断模式的关系,以及在这些标准的发展中持续存在争议的领域。结论:这些基于共识的标准为EMB淋巴细胞性心肌炎的诊断提供了一个标准化的框架。采用这些易于使用的标准将提高淋巴细胞性心肌炎的分类,有助于患者的诊断和治疗。
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引用次数: 0
期刊
Cardiovascular Pathology
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