多普勒超声心动图诊断部分性肺静脉异位引流

目的:评价多普勒超声心动图诊断部分性肺静脉异位引流(PAPVC)的价值。方法:回顾分析132例经心外科手术确诊为PAPVC患者的超声心动图诊断情况。结果:根据Brody分型,132例患者中A型14例,超声诊断11例,诊断正确率78.6%;B型103例,超声诊断57例,正确率55.3%;C型4例,超声诊断3例,诊断正确率75%;D型3例,混合型8例,术前超声均未能做出正确诊断。132例患者中右侧肺静脉异位引流90.1%;左侧肺静脉异位引流7.4%,双侧占2.5%。合并房间隔缺损(ASD)121例(91.7%),其中静脉窦型ASD 40例(33.1%),功能性单心房(大型ASD)或单心房18例(14.9%),继发孔型ASD 63例(52%);9例房间隔完整(6.8%),2例冠状静脉窦隔缺损(1.5%)。结论:彩色多普勒超声心动图可以清晰显示肺静脉的回流情况,准确诊断PAPVC。     

Computed tomography of partial anomalous pulmonary venous connection in adults

OBJECTIVES: To systematically describe the imaging features and clinical correlates of a partial anomalous pulmonary venous connection diagnosed on computed tomography (CT) in adults. METHODS: Twenty-nine adults with a partial anomalous pulmonary venous connection on CT were retrospectively identified. There were 19 women and 10 men, with a mean age of 53 (range: 19-83) years. Four cases were identified by review of 1825 consecutive chest CT reports from July 2000-July 2001, and 25 cases were culled from chest radiology teaching files at 3 institutions. Inclusion criteria were availability of CT images and medical charts. Chest radiographs (25 of 29 cases) were reviewed for mediastinal contour abnormalities, heart size, and pulmonary vascular pattern. Chest CT scans were reviewed for location, size, and drainage site of the anomalous vein; presence or absence of a pulmonary vein in the normal location; cardiac size and configuration; and pulmonary vasculature. Charts were reviewed for evidence of pulmonary and cardiovascular disease, history of congenital heart disease, and results of other cardiac imaging. RESULTS: The prevalence of a partial anomalous pulmonary venous connection was 0.2% (4 of 1825 chest CT reports). Seventy-nine percent (23 of 29 patients) had an anomalous left upper lobe vein connecting to a persistent left vertical vein, only 5% (1 of 23 patients) of whom had a left upper lobe vein in the normal location. Seventeen percent (5 of 29 patients) had an anomalous right upper lobe vein draining into the superior vena cava, 60% (3 of 5 patients) of whom also had a right upper lobe pulmonary vein in the normal location. One patient (3%) had an anomalous right lower lobe vein draining into the suprahepatic inferior vena cava. Chest radiographic findings were abnormal left mediastinal contour in 64% (15 of 25 patients), abnormal right mediastinal contour in 8% (2 of 25 patients), and cardiomegaly in 24% (6 of 25 patients). Computed tomography findings were cardiomegaly in 48% (14 of 29 patients), right atrial enlargement in 31% (9 of 29 patients), right ventricular enlargement in 31% (9 of 29 patients), and pulmonary artery enlargement in 14% (4 of 29 patients). Pulmonary or cardiovascular symptoms were present in 69% (20 of 29 patients), 55% (11 of 20 patients) of whom had specific alternative diagnoses (excluding congestive heart failure and pulmonary hypertension) to explain the symptoms. Only 1 patient (3%) was diagnosed with a secundum atrial septal defect. CONCLUSIONS: A partial anomalous pulmonary venous connection was seen in 0.2% of adults on CT. In contrast to previous series focusing on children, the anomalous vein in adults was most commonly from the left upper lobe, in women, and infrequently associated with atrial septal defects.     

二尖瓣闭锁的MRI诊断

目的 分析二尖瓣闭锁（MA）的病理改变和MRI表现,方法 回顾分析5例经手术证实MA的MRI表现,并与心血管造影（CAG）比较,结果 显示了3型MA的病理改变：双心室左侧房室瓣无孔1例,右室型单心室左侧房室无连接3例,以及左室型单心室左侧房室瓣无孔1例,主要合并畸形有：房间隔膨出瘤1例,全肺静脉异位引流心上型1例,左肺动脉缺如1例及肺动脉狭窄4例,5例行右室和（或）右房造影,除1例外均难以显示MA解剖结构,结论 MRI在MA的诊断中有一定的临床应用价值。     

MSCTA对肺静脉异位引流的诊断价值

目的：比较多层螺旋CT血管成像（MSCTA）和经胸超声心动图（TTE）对肺静脉异位引流的诊断价值。方法：对2007年9月～2009年5月22例临床怀疑先天性肺静脉异位引流的患儿行MSCTA检查及TTE检查,并与手术结果相对照。结果：经手术证实22例病例中,完全性肺静脉异位引流13例,其中心上型6例,心内型5例,心下型2例;部分肺静脉异位引流9例,其中心上型8例,心内型1例。MSCTA和TTE的诊断符合率分别为96%和73%。结论：MSCTA是诊断先天性肺静脉异位引流的重要手段。     

先天性肺静脉异位连接的电子束CT诊断

目的 探讨电子束ＣＴ对肺静脉异位连接的诊断价值。方法 对１４例ＥＢＣＴ诊断的肺静脉异位连接进行回顾性分析。ＥＢＣＴ检查采用增强单层容积扫描,层厚为３ｍｍ,扫描时间为１００毫秒,所用对比剂为非离子型碘对比剂。所有患者均行表面阴影显示法进行三维重建。所有病例均行超声心动图检查。其中８例行心血管造影检查,有２例尚有手术病理对照。结果ＥＢＣＴ诊断完全型肺静脉异位连接１０例,其中心上型６例,心脏型４例;心血     

多层螺旋CT在小儿肺静脉畸形引流诊断中的价值

目的　探讨多层螺旋CT(MSCT)对小儿肺静脉畸形引流的诊断价值。方法　对20例MSCT诊断为小儿肺静脉畸形引流的病例进行回顾性分析。年龄11d至12岁,MSCT采用对比增强容积扫描,层厚1 250mm,层间距0 625mm,均行多平面重建(MPR)、薄层最大密度投影(STS MIP)、容积再现(VR)及表面阴影成像法(SSD)重建。所有病例均行超声心动图(US)检查,其中12例行心血管造影(CAG)检查, 14例尚有手术结果对照。结果　MSCT诊断完全型肺静脉畸形引流9例,部分型肺静脉畸形引流11例。心上型5例、心脏型10例、心下型4例、混合型1例。MSCT均清晰显示异常连接肺静脉的数目、分布和位置。CAG漏诊3例,US漏诊10例。14例有手术对照者,MSCT对肺静脉畸形引流的定性、定位诊断全部正确。结论　MSCT较常规CAG及US可明显提高小儿肺静脉畸形引流的检出率,对本病有重要的诊断价值。     

Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants

OBJECTIVE: The objective of this study was to investigate the usefulness of helical CT angiography in the evaluation of total anomalous pulmonary venous connections. MATERIALS AND METHODS: Fourteen patients with total anomalous pulmonary venous connections underwent helical CT angiography and subsequent three-dimensional (3D) reconstruction. They ranged in age from 3 days to 8 months (median age, 2.3 months) and in weight from 2.3 to 7.1 kg (median weight, 4.3 kg). The types of total anomalous pulmonary venous connections and the number of pulmonary veins were evaluated on axial and 3D images. Qualitative evaluations were performed for extent of pulmonary vascular enhancement and contrast- or motion-induced artifacts. RESULTS: In all patients, helical CT angiography correctly depicted total anomalous pulmonary venous connections. Seven cases were the supracardiac type, four cases were the cardiac type, one case was the infracardiac type, and two cases were the mixed type. The detection rate of the pulmonary vein in 3D reconstruction images (95-98%) was slightly lower than that of the pulmonary vein in the axial images (100%), but the difference between axial and 3D reconstruction images was not statistically significant (p > 0.1). No statistically significant differences were noted among 3D reconstruction images in the detection rates of the pulmonary vein (p > 0.1). The extent of contrast enhancement of the pulmonary vein was good or excellent in all patients. In five patients, there were contrast-induced artifacts that made some surrounding vascular distortion but did not interfere with the pulmonary vein analysis, except in one patient. Motion-induced artifacts were observed in nine patients. One of them had an obstacle in pulmonary vein analysis. CONCLUSION: The combination of axial and 3D images in helical CT angiography is helpful in the assessment of a total anomalous pulmonary venous connection containing the individual pulmonary vein, and this combination can be a good diagnostic tool in preoperative evaluation of neonates and infants with a total anomalous pulmonary venous connection.     

完全性心上型肺静脉畸形引流的治疗

目的 探讨完全性心上型肺静脉畸形引流的治疗方法。方法 手术治疗3例,在全麻体外循环下行矫治术。2例采用联合法,1例采用心上法。结果 3例均存活,无远期死亡。结论 心上法矫治完全性心上型肺静脉畸形引流可获得良好的显露和足够大的吻合口,手术对心脏的损伤小,可降低术后心律失常的发生率。     

电子束CT在肺静异常连接诊断中的临床价值

目的 探讨电子束CT（EBCT）在肺静脉异常连接诊断中的临床价值。材料与方法 EBCT诊断肺静脉异常连接患者21例,其中男13例,女8例。年龄3－63岁,平均15.6岁。17例行增强单层容积扫描,4例行增强连续容积扫描。全部病例均行表面阴影显示法三维重建。17例有心血管造影或手术结果,其中9例单纯行造影,4例单纯行手术,4例造影后手术。结果 20例术前资料,EBCT诊断为完全型肺静脉异常连接12例,部分型肺静脉异常连接8例,前者包括心上型3例,心脏型8例和混合型1例。EBCT均清晰显示异常连接肺静脉的数目、分布和位置。1例术后复查病例,ERCT示吻合口狭窄。7例完全型肺静脉异常连接者,EBCT提示合并内脏心房异位综合征。8例有手术对照者,EBCT对肺静脉异常连接的定性、定位诊断全部正确。结论 EBCT及其三维重建可准确显示异常连接 肺静脉的位置、数目和术后狭窄,对明确胸腹并发畸形有明显优势,是一种重要的无创性诊断肺静脉异常连接的方法。     

Scimitar syndrome: MDCT imaging revisited

Scimitar syndrome refers to a form of anomalous pulmonary venous drainage which implies partial or total failure of the pulmonary veins to reach the left atrium.AimTo emphasize the role of MDCT in the diagnosis of Scimitar syndrome.Patients and methodsWe presented MDCT imaging findings in three patients with Scimitar syndrome.ResultsMDCT revealed anomalous arterial blood supply from abdominal aorta to right lower lung lobe and anomalous venous drainage into infradiaphragmatic IVC, hypoplastic right pulmonary artery and hypoplastic right lung as well as atrial septal defect and bronchial diverticulum.ConclusionMDCT is essential investigation in Scimitar syndrome.     

Abnormalities of the pulmonary veins: evaluation with MR imaging and comparison with cardiac angiography and echocardiography

Seventy-seven patients underwent T1-weighted spin-echo magnetic resonance (MR) imaging. Group 1 (n = 56) consisted of patients with various types of congenital heart disease but normal pulmonary veins. Group 2 (n = 22) consisted of patients with the following conditions: partial anomalous pulmonary venous connection (n = 11), total anomalous pulmonary venous connection (n = 5), cor triatriatum (n = 4), or pulmonary vein stenosis (n = 2). In group 1, the sites of connections of all four pulmonary veins were identified with MR imaging in 88% of cases; the connections of at least three pulmonary veins were seen in all patients. In group 2, the prospective detection rate of pulmonary venous abnormalities with MR imaging was 95%. The prospective detection rates of pulmonary venous abnormalities with cardiac angiography (n = 13) and echo-cardiography (n = 13) were 69% and 38%, respectively. This study indicates that MR imaging can accurately demonstrate the normal pulmonary veins and abnormalities of the pulmonary veins.     

Anomalous intrapulmonal vein drainage and pulmonary vein connection in DSA

Anomalous pulmonary venous drainage can be detected and evaluated by angiographic studies. Now that intravenous DSA is available, this method can be used to demonstrate pulmonary venous return. This study presents the results of DSA examination in three patients having anomalous pulmonary vein connection with the left innominate vein and the superior vena cava respectively. In a fourth patient, intra-pulmonary atypical venous return could be demonstrated by DSA without anomalous connection. This minimal invasive procedure is diagnostically useful in anomalies of pulmonary veins when recommended post-processing techniques are evaluated, particularly in patients with additional cardiac septal defects.     

Unsuccessful lung scan due to major right-to-left shunt through a sinus venosus septal defect

In a patient with a prior history of cerebral abscess and cerebral ischemia, an unsuccessful perfusion lung scan led to a radionuclide angiocardiogram using an arm vein injection. This showed a total right-to-left (R-L) shunt from the superior vena cava (SVC) to the left atrium. Repeat radionuclide study, through a leg vein, demonstrated a moderate R-L shunt and an interpretable lung scan could be obtained. Catheterization and contrast cineangiogram did not provide the exact diagnosis, the preoperative conclusion being anomalous drainage of the SVC into the left atrium, with atrial septal defect (ASD) and partial anomalous pulmonary venous connection to the SVC. The operative diagnosis was high atrial (sinus venosus) septal defect. This example of major but clinically unsuspected R-L shunt emphasizes the value of performing a perfusion lung scan, preferably in conjunction with radionuclide angiocardiography in patients with a prior history of unexplained cerebral abscess or systemic ischemia. Implications of the site of an ASD on quantitation of L-R shunts by radionuclide methods are also discussed.     

双源CT对先天性心脏病肺静脉异常连接的诊断价值

目的：探讨双源CT（DSCT）在先天性心脏病肺静脉异常连接（APVC）中的诊断价值。方法：35例患者中男25例,女12例。22例经手术证实,对DSCT诊断APVC类型进行分析。结果：双源CT诊断APVC的符合率为100%（22/22）,对伴发畸形的诊断符合率为90.9%（20/22）。DSCT诊断完全型APVC 20例57.1%（20/35）,最常见异常连接血管为上腔静脉48.9%（23/47）、垂直静脉42.6%（20/47）,多数存在伴发畸形95%（19/20）,房间隔缺损最常见52.6%（10/19）。DSCT诊断部分型APVC 42.9%（15/35）,最常见异常连接为右肺静脉连接至右心房占51.6%（16/31）,多数存在伴发畸形86.7%（13/15）,房间隔缺损最常见46.2%（6/13）。结论：DSCT可以满足APVC的诊断。     

Acquired anomalous intrapulmonary venous connection secondary to pulmonary venous stenosis

An unusual case of acquired development of anomalous intrapulmonary venous connection with pulmonary venous stenosis is presented. Appearances on a chest radiograph resembled the "scimitar" sign in a patient with previous surgery for partial anomalous pulmonary venous return. Spiral CT and pulmonary arteriography showed stenosis of the right upper pulmonary vein and an anomalous intrapulmonary venous connection between the right upper pulmonary vein and the right lower pulmonary vein. We consider the slow progression of pulmonary vein stenosis led to anomalous intrapulmonary venous connection as an intrapulmonary collateral.     

MR imaging of partial anomalous pulmonary venous connections

Magnetic resonance imaging was performed on 11 patients with partial anomalous pulmonary venous connections (PAPVC). Ten of these patients also had echocardiographic examinations, eight of which included color-flow Doppler studies. The diagnosis of PAPVC was confirmed in each of these patients by surgery or angiography. Fourteen anomalous pulmonary venous connections were identified, 10 involving the right upper lobe pulmonary vein and 4 involving the left upper lobe pulmonary vein. This retrospective review demonstrated that all 14 anomalous venous connections were correctly identified by MR imaging, whereas only 8 of 13 (62%) were identified by echocardiography. With MR, 89% of all the pulmonary veins and 93% of the anomalous pulmonary veins were visualized on axial images, while 41% of all pulmonary veins and 71% of anomalous veins were seen on coronal MR images. There were five atrial septal defects (ASDs), four of the sinus venous type and one of the septum secundum type. All five ASDs were correctly identified with MR imaging; three of four ASDs were identified with echocardiography. We conclude that MR imaging provides an accurate noninvasive method of depicting the anatomic abnormalities associated with PAPVC.     

双源CT血管造影及三维重建在肺静脉畸形连接中的临床应用

目的:探讨双源CT血管造影及三维重建对肺静脉畸形连接的诊断价值。方法:对24例手术证实为肺静脉畸形连接的病例进行回顾性分析。所有病例均行双源CT对比增强容积扫描,并进行MPR、CPR、MIP、MinIP、VR等。所有病例均行超声心动图(US)检查,其中11例行心血管造影(CAG)检查。结果:双源CT对肺静脉畸形连接的定性及定位诊断符合24例;超声心动图诊断21例,怀疑1例,心血管造影9例与手术对照符合,怀疑2例。结论:双源CT对肺静脉畸形连接的诊断符合率较高,可准确给予分型,对外科手术方式的选择具有重要意义。     

Partial anomalous pulmonary venous return

Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that occurs in 0.4 to 0.7% of postmortem examinations. Ninety percent of these anomalies are associated with an atrial septal defect. Partial anomalous pulmonary venous return occurs more commonly on the right than the left and is manifested by abnormal return of the pulmonary veins to the central venous circulation. Most patients are asymptomatic, but when symptoms are present they are due to shunting of oxygenated blood to the venous circulation. We submit the case of a recently activated solider who presented with dyspnea on exertion refractory to inhaled corticosteroids and an 8.5-mm solitary pulmonary nodule. Further diagnostic imaging revealed PAPVR. Our case appears to be the first report of a solitary pulmonary nodule as the initial presentation of a right upper lobe PAPVR with return to the superior vena cava in the absence of associated atrial septal defect.     

Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.     

Reliability of MDCT in characterizing pulmonary venous drainage, diameter and distance to first bifurcation: an interobserver study

RATIONALE AND OBJECTIVES: To evaluate the interobserver agreement of readers in evaluating pulmonary venous anatomy and in measuring pulmonary vein ostial diameters and distance to first bifurcation. MATERIALS AND METHODS: This study was approved by our institutional review board. Thin-section contrast material-enhanced multidetector computed tomography examinations of the thorax were retrospectively reviewed in 200 consecutive patients (38 females and 162 males), age 24-79 years (mean, 52.8) referred for imaging before radiofrequency ablation therapy for atrial fibrillation. For each patient, pulmonary venous anatomy and drainage patterns including the number of venous ostia was assessed independently by experienced cardiothoracic radiologists. Pulmonary vein ostial diameter and distance to the first bifurcation of the four major pulmonary veins (right inferior and superior, left inferior and superior), the middle lobe pulmonary vein, and any anomalous pulmonary veins (common trunks and accessory veins) were measured independently at a workstation. Interreader assessment of pulmonary venous anatomy was evaluated using the Kappa statistic. Interreader variation in measurements of venous diameter and distant to first bifurcation were estimated by Bland-Altman plots and Pitman's test of difference in variance. RESULTS: Very good to excellent interreader agreement in detection of anomalous pulmonary venous anatomy, middle lobe pulmonary venous drainage, and other thoracic venous anomalies. No significant variation between readers in pulmonary vein ostial diameter measurements for the four major and middle lobe pulmonary veins, or the anomalous pulmonary veins. Significant interreader variability was noted in measurements of the pulmonary vein distance to first bifurcation for the right inferior (P = .017), middle lobe (P = .005), and left inferior (P = .015) pulmonary veins. CONCLUSIONS: There is excellent interobserver agreement when evaluating normal and anomalous pulmonary venous drainage patterns, and when measuring normal or anomalous pulmonary vein diameters. However, measurements of distances to first bifurcation were less reliable across readers.