目的:探讨永存第五对主动脉弓(PFAA)的解剖学特征,评价几种常用影像学方法的诊断价值.方法:回顾分析2010年10月至2019年9月中国医学科学院阜外医院诊断的8例PFAA患者,收集其临床、影像及手术资料,总结其解剖及影像学特征、类型和伴发畸形情况,对比X线胸片、超声心动图、CT血管造影(CTA)检查结果.结果:本组8例患者中,7例的PFAA与主动脉弓位于同侧、不形成血管环结构,4例的PFAA伴发狭窄及闭锁,8例PFAA均起自升主动脉远端无名动脉开口旁或对侧,6例止于降主动脉近端,2例止于一侧肺动脉干;8例患者均合并心血管畸形,包括室间隔缺损、主动脉弓离断、法乐四联症、动脉导管未闭、共同动脉干、右心室双出口、肺动脉闭锁,以室间隔缺损和主动脉弓离断最常见;CTA对8例PFAA及伴发的其他心血管畸形患者均做出准确诊断,其准确度优于超声心动图及造影结果.结论:PFAA的解剖特征为:起自升主动脉远端无名动脉开口旁或对侧、止于降主动脉近端或一侧肺动脉干,呈弓状血管结构,部分伴发狭窄及闭锁,常合并其他心血管畸形.CTA为PFAA首选的无创检查方法,可部分替代或补充血管造影结果.

The histopathological features of IgG4-related coronary arteritis involve a large number of IgG4-positive plasma cell infiltrates in the tissues of the coronary adventitia, causing adventitia fibrosis and diffuse enlargement, forming a boudoir mass around the coronary artery with neoplastic thickening of the artery wall. CT angiography of the coronary artery shows the thickening of the coronary vessel wall and the mistletoe sign formed by the surrounding tumor-like mass which are the characteristic manifestations of this disease. This article expounds on IgG4-related coronary arteritis and explains its pathogenesis, imaging features and diagnostic criteria.

1 临床资料 患者男性,62岁,主因心慌、胸闷10余天,于2019年8月7日入我院就诊.冠状动脉CT血管成像(CCTA) 检查见图1～8. 图1～8显示:冠状动脉左主干、前降支、右冠状动脉钙化及非钙化斑块,管腔中～重度狭窄,左主干、前降支、回旋支、右冠状动脉节段性管壁增厚,管腔周围软组织密度影环绕,冠状动脉血管束上周围肿物生成,呈多发"肉肠卷"或"槲寄生"样改变.

Poster: ECR 2019 / C-2883 / CT diagnosis and prognosis in patients with aortic dissection or intramural haematoma type A involving pulmonary artery by: A. Zhi , R. Dai, P. Zhang, S. Jiang, B. Lv; Beijing/CN

2015年曾在阜外医院见到过这样一个病例,起初从临床表现看毫无特色,但从移植取下来的心脏看却十分奇特、罕见,我们查阅国内外文献鲜见报道,通过回顾、追索和探讨得到了许多有益启示.依次从临床、病理就可以看出该病例的病变特征、临床表现的病理基础,从病变部位可以解释该病例临床表现为限制型心肌病的原因,经过一步步分析才追索到其病源.

Objective: Previous studies assessing left atrium (LA) function in patients with atrial fibrillation (AF) have not simultaneously analyzed left ventricle (LV) function.To evaluate differences in LA and LV volume and function in patients with AF during and between AF episodes compared to healthy subjects by using dual-source computed tomography (DSCT).Materials and Methods: Sixty-one consecutive patients with AF and 30 healthy subjects underwent retrospective electrocardiography-gated DSCT.Thirty of 61 AF patients experienced AF during scan acquisition (AF episode subgroup), and 31 were in sinus rhythm (AF non-episode subgroup).LA and LV functions were analyzed with dedicated software and groups were compared with analysis of variance and least significant difference test. Results:The maximal LA volumes in healthy subjects, the AF episode subgroup, and the no AF episode subgroup were 94.4±19.9mL, 121.8±42.4mL, and 101.4±32.9mL, respectively (p=0.005).Left ventricular end-diastolic volumes (LVEDV) in healthy subjects, the AF episode subgroup and the no AF episode subgroup were 160.5±34.4mL, 131.7±37.3mL, and 138.1±27.6 mL, and respectively (p=0.003).Left ventricular end-systolic volumes were 55.6± 19.4 mL, 65.2±32.4mL, and 51.0±14.9mL, respectively (p=0.060).Increased LA volume, decreased LVEDV, and decreased LA and LV systolic function were found during AF episodes. Conclusion:Our data contributes to understanding the mechanism of AF and guides clinical management of patients with AF.

Objective: To explore the imaging and clinical features of Behcet's disease with coronary artery involvement in order to improve the diagnosis and treatment in clinical practice. Methods: A total of 6 Behcet's disease patients with coronary involvement diagnosed and treated in our hospital from 1999-08 to 2016-11 were analyzed. Coronary angiography (CAG) and coronary CTA were performed for diagnosis. There were 5 cases received CAG and 5 cases received CTA examinations. Results: All 6 patients had the first clinical visit by sudden pericardial tapenade, myocardial infarction or cardiac shock. For diagnosis: the imaging presentation included coronary stenosis, occlusion and pseudo aneurysm formation. 3 patients had anterior descending artery involvement, 1 had the far-end of left circumflex involvement and 2 had the middle segment of right coronary involvement; all patients were combining large amount of pericardial effusion. For treatment: there were 3 patients with aneurysm received conservative treatment (1 case had open thoracic exploration), 1/3 lost contact and 2 having long-term normal life; 3 patients received coronary stent implantation without optimal effect. Conclusion: The patients of Behcet's disease with coronary involvement may easily form pseudo aneurysm; CAG/CTA has been helpful for diagnosis. Clear pre-operative diagnosis of etiology is important for treatment.

临床医学的进步离不开医学影像设备的发展和应用技术水平的提高。计算机断层摄影术（CT）冠状动脉血管成像应用短短十年，已经成为门诊筛查、排除或者确诊冠心病的重要工具。近年CT心肌灌注成像（CTP）和CT血流储备分数（CT-FFR）等功能学CT影像技术迅速推出，并获得重要循证医学研究证据，临床广泛关注，被认为具有改变临床诊断和治疗路径、优化冠心病诊疗策略和改善预后的作用。本文对这些新技术的现状和前景做一简要介绍，以期尽快在我国获得临床应用。

OBJECTIVE:To explore impact of coronary CT angiography findings on preventive medical treatment and control of coronary artery disease (CAD) risk factors.METHODS:Consecutive patients with atherosclerotic plaque detected by coronary CT angiography were enrolled in our study from September 2013 to December 2014, grouped as <50% stenosis and ≥ 50% stenosis.Baseline and follow-up data were recorded.Comparative analysis was performed both between stenosis groups and pre- and post-CT angiography data.Multivariable Logistic regression were preformed to investigate association between coronary CT angiography findings and subsequent medical therapies.RESULTS:Totally 160 patients were enrolled in our study, 99 were <50% stenosis and 61 were ≥ 50% stenosis.Significant reduction of total cholesterol (5.06 ± 1.04 vs 4.54 ± 1.09 mmol/L, P<0.01), low-density lipoprotein cholesterol (3.16 ± 0.95 vs 2.60 ± 0.88 mmol/L, P<0.01), and triglyceride (1.66 (1.14, 2.28) vs 1.55(1.07, 2.05) mmol/L, P=0.004) were observed Pre- versus post-CT angiography. Compared to patients with <50% stenosis, patients with ≥ 50% stenosis demonstrated more significant reduction with regard to total cholesterol (-0.70 ± 0.94 vs -0.42 ± 0.96 mmol/L, P=0.035) and low-density lipoprotein cholesterol (-0.78 ± 0.99 vs -0.43 ± 0.79 mmol/L, P=0.016). After CT angiography, aspirin (13.8% vs 65.6%, P<0.01) and statin (20.0% vs 71.9%, P<0.01) use were significantly increased, blood pressure medication (53.1% vs 63.1%, P=0.07) use showed no statistical differences. Adjusted for baseline risk factors and pretest medications, CT angiography findings were independently associated with increased post-CT angiography use of aspirin (adjusted OR (95% CI) : 3.58 (1.61-7.99), P=0.002) and statin (adjusted OR (95% CI) : 15.01 (4.40-51.22), P<0.01).CONCLUSION:Coronary CT angiography findings demonstrated direct impact on subsequent medical therapies and control of CAD risk factors, and offered important guidance for prevention strategies of CAD.

Objective: To evaluate the clinical characteristics in patients with coronary sinus oriifce atresia (CSOA). Methods: We retrospectively studied 22 CSOA patients for their clinical characteristics of cardiovascular involvement. They were treated in our hospital from 2005-02 to 2014-05 including 15 male and 7 female at the age of 1.5-76 (50.88 ± 14.97) years. All patients receive echocardiography and coronary CTA examination. Results: The diagnosis of CSOA was confirmed by contrast-enhanced cardiac MDCT in all patients. According to coronary vein blood flow direction, the patients were classified into 3 types: LA (left atria) type,n= 9 patients with blood flow back into LA including 3 cases from left superior vena cava (LSVC) to LA and 2 combining with complicated congenital cardiac abnormalities. RA (right atria) type,n=5 patients with blood flow back to RA including 2 cases directly to RA and 3 cases indirectly from LSVC to RA. Mixed type,n=8 patients with small branch of blood flow back to both LA and RA including 4 cases combining LSVC involvement. There were 9/22 patients with LSVC involvement including 3 with blood flow directly to LA and 6 with blood flow via in-nominate vein to RSCV then to RA. Echocardiography could not diagnose CSOA. ECG showed 5 patients with arrhythmia and 3 of them had successful RFCA, 1 failed RFCA and 1 received permanent pace maker implantation. There were 6/22 patients combining congenital cardiac abnormalities including 1 of atrial septal defect (ASD), 5 of complicated congenital heart disease (CHD), and 3 of them with confirmed CSOA diagnosis by contrast-enhanced MDCT before operation, 2 having post operative symptom and the diagnosis was confirmed by CT. All 5 CHD patients received operation and no one had operation for CSOA alone. Conclusion: Clear diagnosis of CSOA and its associated arrhythmia, LSVC involvement and cardiac abnormalities are important for preventing cardiovascular accident. Contrast-enhanced MDCT has the important value for diagnosing the patients with CSOA.

目的:评价家族性高胆固醇血症(FH)累及心血管系统的临床特点.方法:回顾分析2002-01至2013-11我院经临床诊断为FH的14例患者,其中男性10例,女性4例,年龄8～39[(平均(24.43±9.88)]岁.14例患者均行超声心动图检查,11例患者行冠状动脉计算机断层摄影术(CT)增强扫描或冠状动脉造影检查.结果:14例患者均有黄色瘤和(或)腱黄瘤,其中10例为纯合子,4例为杂合子.病变累及左主干10例、前降支8例、回旋支4例、右冠状动脉9例.超声心动图检查显示,14例患者中6例左心室壁普遍肥厚,6例左心室增大,4例左心室功能减低.所有患者主动脉均呈粥样硬化性改变,13例表现为主动脉根窦部管壁明显增厚、钙化斑块形成;7例患者主动脉瓣受累;10例主动脉瓣上狭窄;8例降主动脉和(或)腹主动脉管腔不规则、多发狭窄;1例表现为主动脉全程明显扩张,降主动脉、腹主动脉瘤及大量附壁血栓形成.1例患者行冠状动脉支架置入术,共5例患者行冠状动脉旁路移植术,2例行主动脉瓣上狭窄矫正术(其中1例患者同时行冠脉旁路移植手术).结论:FH患者早发心血管系统受累,早期明确病变程度及特点为临床提供治疗依据,对预防心血管意外的发生有重要意义.

PURPOSE:To compare coronary plaque burden, composition, distribution and the degree of coronary artery stenosis in invasive coronary angiography (ICA) diagnosed coronary artery disease (CAD) patients with or without metabolic syndrome (MetS).METHODS:From January 2008 to June 2011, consecutive patients underwent both coronary computed tomography angiography (CCTA) and ICA within three months were enrolled. Patients with history of previous percutaneous coronary interventions (PCI) and coronary artery bypass grafting (CABG) were excluded. Plaque characteristics and maximal luminal diameter stenosis were analyzed on a 16-segment basis as suggested by the American Heart Association classification.RESULTS:The study population consisted of 872 patients [age (60.2 ± 10.0) years, 72.70% males] including 377 patients with MetS and 495 patients without MetS. The median coronary artery calcium score (CACS) was higher in MetS patients than in non-MetS patients [102 (10, 410) vs. 58 (0, 274) , P < 0.01]. Percentage of patients with no coronary artery calcium was significantly lower in MetS group than in non-MetS group [19.63% (74/377) vs. 30.71% (152/495) , P < 0.01], while percentage of patients with severe coronary calcium (CACS ≥ 1000) were significantly higher in MetS than non-MetS group [8.22% (31/377) vs. 4.65% (23/495) , P = 0.03]. The proportion of patients with 1-vessel disease was lower [23.61% (89/377) vs. 36.77% (182/495), P < 0.01], 2-vessel [29.71% (112/377) vs. 22.83% (113/495), P < 0.05] and 3-vessel disease [35.54% (134/377) vs. 24.44% (121/495) , P < 0.01] were higher in MetS group than in non-MetS group. Calcified plaque of LM and the middle and distal coronary artery were significantly higher in MetS group than in non-MetS group (all P < 0.05) .CONCLUSIONS:CAD patients with MetS are associated with severer coronary artery calcium deposition and higher percentage of calcified plaque in the middle and distal coronary arteries and severer obstructive coronary vessels.

目的:评价非医源性冠状动脉假性动脉瘤的影像学诊断及临床特点,以期提高临床诊治.方法:分析1999-08至2013-06阜外心血管病医院诊治的9例非医源性冠状动脉假性动脉瘤患者.病因包括粥样硬化病变(4例),Behcet's综合征(3例),先天性冠状动脉瘤破裂(1例),不明原因(1例).9例均行冠状动脉CT血管造影(CTA)检查.7例患者行冠状动脉造影,结果:9例非医源性冠状动脉假性动脉瘤累及前降支5例,回旋支2例,右冠状动脉2例.2例患者出现心包积液.2例患者冠状动脉旁路移植手术(1例为特发性,另1例为先天性);心脏移植1例(粥样硬化性);开胸探查手术1例(Behcet’s综合征);3例保守治疗(2例为Behcet's综合征患者,另1例为粥样硬化性病变);1例随诊观察;1例失访.本组患者无冠状动脉介入治疗者.结论:非医源性冠状动脉假性动脉瘤临床症状无特异性,通过冠状动脉造影和(或)冠状动脉CTA可以确诊.术前明确病因诊断对临床治疗方法选择有重要意义.治疗方法可以采用保守、介入或外科旁路移植术手术治疗.

肺动脉栓塞(又称肺栓塞)是常见的心血管病,死亡率达20％～30％,在西方国家仅次于肿瘤和冠心病居第三位.在我国虽然没有确切的流行病统计报告,但并不少见.绝大部分肺动脉栓塞生前未能得以正确诊断,根据国内外尸检报告,这一数字高达67％ ～ 79％.如能及时诊断治疗,病死率可以下降5～6倍.误、漏诊的主要原因是医生对该病的认识不足,其次是缺乏合理地应用检查手段和正确认识检查结果.肺动脉栓塞病人首诊主诉或主要症状表现出多样化,轻症者无任何症状,重症者可发生呼吸困难,导致心源性休克.影像学检查是确诊的主要手段.

患者女,46岁.活动后胸闷乏力1年,近1个月加重,于2009年12月18日就诊.体检:主动脉瓣听诊区和主动脉瓣第二听诊区可闻及Ⅱ～Ⅲ级收缩期杂音,呈喷射样,向颈部传导.心电图提示左心室高电压,ST-T改变.超声心动图示先天性心脏病,主动脉瓣二瓣化畸形并重度狭窄,升主动脉扩张.X线摄影示两肺纹理重,升主动脉扩张,主动脉结增宽,左心室增大,心胸比为0.63。

Objective To evaluate the effectiveness of MSCT in the diagnosis of superior sinus venosus atrial septal defect.Methods The MSCT features of superior sinus venosus atrial septal defect in twenty cases were evaluated retrospectively.The following data were recorded:the size and location of sinus venosus atrial septal defect,the anatomy of pulmonary vein,including number of anomalously draining pulmonary veins and their site of drainage,and associated anomalies.Results In all patients,the superior sinus venosus atrial septal defect locates in the extraseptal wall,which normally separates the right upper pulmonary vein from superior vena cava(SVC).And anomalous connection of right upper pulmonary venous and SVC was identified in all the patients.The mean value of the defect diameter was ( 17.1±5.8) mm.Left superior vena cava was identified in 3 patients.In an elderly patient,left anterior descending branch of coronary artery presented significant stenosis.And in another elderly patient with large atrial septal defect,severe pulmonary hypertension was identified by cardiac catheterization.MSCT findings of superior sinus venosus atrial septal defect in 6 cases were finally confirmed by surgical operation.Conclusions Contrastenhanced MSCT was a useful technique for the diagnosis of superior sinus venosus atrial septal defect,which accurately displayed the anatomical characteristics of the associated malformations for preoperative evaluation.

<正>近年来,随着先天性心脏病(简称先心病)外科手术、介入及护理技术的进步,越来越多的先心病患者可行早期手术,心血管影像诊断的需求也随之提高,先心病的检查有多种,不同的方式各有优缺点,如何合理选择不同的检查方式,以达到程序简洁、结果明确,是我们广大医学影像工作者应该关心的问题。一、先心病概述1.先心病的流行病学:据WHO统计资料显示,全球每年约

Objective To evaluate the CT features of aorto-right atrial fistula after aortic valve replacement(AVR) or ascending aortic replacement.Methods Eighty-seven patients with aortic-right atrial fistula underwent CT after operation.The CT features were retrospectively analyzed.Fistula was measured according to maximum width of the shunt.Results Aorto-right atrial fistula was detected in 87 patients after aortic valve replacement or ascending aortic replacement by CT scan. Among them,25 patients were diagnosed as mild aorto-right atrial fistula,47 patients as moderate,and 15 patients as severe.Thirty-seven patients underwent follow-up CT.Among them,10 patients with mild to moderate aorto-right atrial fistula were considered to have complete regression,8 patients with mild aorto-right atrial fistula considered to have incomplete regression,14 patients with mild to moderate aorto-right atrial fistula considered to have stable condition,and 5 patients with moderate aorto-right atrial fistula considered to have progression at the 3-month follow-up.Conclusion CT is a useful tool for defining aorto-right atrial fistula after AVR or ascending aortic replacement and for evaluating it in follow-up.

Objective To investigate the intra- and interobserver repeatability of coronary artery disease (CAD) diagnosis based on invasive coronary angiography (ICA) and CT coronary angiography (CTCA).Methods Two readers with comparable experience ( over 10 years) independently evaluated ICA results of 42 consecutive patients with a blind method. After 30 days,one of them reviewed the same patients again.Another two comparable-experience (over 10 years) readers evaluated the results of CTCA (prospectively ECG-triggering) from the same 42 patients in the same way.The inter-reader and intra-reader repeatability of ICA and CTCA were analyzed by performing Kappa test and calculating the percentage of the segments with agreement on stenotic degree.Using ICA as reference,the accuracy of CTCA in diagnosing CAD was studied by comparing the area under ROC. Results The Kappa between readers for ICA and CTCA were 0.91 and 0.81.Intra-reader Kappa were 0.92 and 0.83 respectively (x2 =509.4 and 432.5,all P ＜0.01 ).The percentage of the segments with agreement between readers on the degree of stenosis were 80.8％ (494/611) in ICA and 75.2％ (469/624) in CTCA ( x2 =2.75,P =0.10),and within the same reader,86.9％ (531/611)in ICA and 81.9％ (511/624) in CTCA(x2 =3.76,P =0.053).With≥ 50％narrowing as a CAD diagnosis criterion,the agreement rates for two readers were 96.6％ (590/611 ) in ICA and 94.4％ (589/624) in CTCA( x2 =3.36,P =0.07),and for the same reader,97.4％ (595/611) in ICA,95.4％ (595/624) in CTCA ( x2 =3.62,P =0.06).Using ICA as reference,two readers of CTCA results achieved a sensitivity and specificity of 84.9％ (530/624)and 98.1％ (612/624).The area under ROC was 0.94 (95％ CI 0.91-0.97).Conclusions Both ICA and CTCA demonstrate good repeatability in diagnosing CAD.The repeatability of ICA is superior to that of CTCA.A certain discrepancy exists in two readings from the same reader or two readers.

OBJECTIVE:To analyze the diagnostic feature, treatment and prognosis of patients with Cantrell syndrome.METHODS:Clinical manifestation, diagnosis, operation and follow-up data of 5 patients with Cantrell syndrome were summarized in this retrospective analysis.RESULTS:The age of the 5 patients was 7 days-76 years, definite diagnosis was made in 3 cases and 2 cases presented feature of incomplete Cantrell syndrome. Three patients with full Cantrell syndrome were correctly diagnosed before operation and confirmed by operation. One patient with incomplete Cantrell syndrome (two-vessel stenosis) received bypass surgery. Another asymptomatic patient with incomplete Cantrell syndrome (apical diverticulum of the left ventricle) does not need operation and is under observation. During follow-up, 1 patient died at 60 months after operation and the remaining 4 patients are alive and well.CONCLUSIONS:With the development of modern imaging technology, it becomes easy to make correct diagnose Cantrell syndrome before operation. Prognosis is fine post timely operation and related intervention.