The various forms of pulmonary varices. Report of three cases and review of the literature.

Ture pulmonary varices are congenital local dilatations of a pulmonary vein or veins, with normal or collateral transpulmonary drainage into the left atrium. Pulmonary varices do not cause pulmonary venous hypertension, but existing varices can further become dilated by pulmonary venous hypertension due to mitral valve disease. Embryologically, pulmonary varices may represent residual primitive splanchnic venous components incorporated into the pulmonary venous system, or atresia of an individual pulmonary vein which occurred at a time that enable adoption of unobstructed transpulmonary collateral drainage into the left atrium. True pulmonary varices do not produce symptoms, do not change in diameter over the years, and, usually, do not require treatment.     

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.     

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.     

3D multidetector CT angiographic evaluation of extralobar pulmonary sequestration with anomalous venous drainage into the left internal mammary vein in a paediatric patient

Pulmonary sequestration is a congenital lung malformation, defined by dysplastic and non-functioning lung tissue lacking normal tracheobronchial connections and accompanied by an anomalous systemic blood supply. Recognition of anomalous arteries and veins in pulmonary sequestration is paramount to making the correct diagnosis. In contrast to intralobar pulmonary sequestration, where anomalous venous drainage is usually into the pulmonary venous system, the pattern of anomalous venous drainage is more varied in extralobar pulmonary sequestration. To the best of our knowledge, anomalous venous drainage to the internal mammary vein in extralobar sequestrations has not been reported. We report an anomalous venous drainage into the internal mammary vein in an extralobar sequestration which was evaluated with 3D multidetector CT angiography.     

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.     

Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome

A case in which anomalous systemic venous drainage occurred in association with the hypogenetic lung syndrome (scimitar syndrome) is described. The chest radiograph appearances of the anomalous systemic vein mimicked an anomalous pulmonary or scimitar vein. Angiography demonstrated that the patient also had a small anomalous pulmonary vein draining and a systemic artery supplying, the right lung. As the right lung was hypoplastic, all three features of the hypogenetic lung syndrome were present, in addition to partial anomalous systemic venous drainage.     

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.     

主动脉弓左侧先天性静脉异常的CT鉴别诊断

目的：分析41例主动脉弓左侧先天性静脉变异的CT表及鉴别诊断。方法：所有患者均有CT平扫,其中3例行增强扫描,5例另做MRI或超声检查确诊,6例做最大信号强度投影和表面遮盖法三维重建。结果：永存在上腔静脉24例,其中双上腔静脉23例,永存左上腔静脉并右上腔静脉缺如1例,异常左头臂静脉11例,其中6例合并主动脉畸形;左上叶的部分异常肺静脉引流4例,其中1例并双上腔静脉畸形;左头臂静脉与奇静脉异常连接2例,它们的共同表现是在主动态弓可见圆形或类圆形结影,但它们具有各自不同的血管行程,可加以区别。结论：熟悉这些少见的先天性异常有助于避免误诊,高质量的DT增强扫描这些先天性异常的诊断及鉴别诊断有重要价值。     

Anomalous pulmonary venous connection of entire left lung with intact atrial septum.

Three cases of total unilateral pulmonary venous connection of the left lung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein in the superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar to what is seen in cases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the left pulmonary veins and documented the absence of an atrial septal defect.     

Significance of the site of injection in unexpected right-to-left shunting

Lung perfusion scintigraphy, with Tc-99m MAA injected into a left antecubital vein, revealed extensive uptake in the myocardium, kidneys, spleen, thyroid, and brain. After a right antecubital injection, a normal pulmonary perfusion pattern was found. This finding is specific for right-to-left shunting due to anomalous systemic venous drainage into the left atrium.     

Scimitar syndrome – An incidental diagnosis in a case of fibroadenoma

Scimitar syndrome is a rare congenital anomaly which is characterised by anomalous pulmonary venous drainage of the either entire right lung or part of it into the inferior vena cava or portal vein or hepatic vein or right atrium occasionally. This can be associated with hypoplasia of the right lung, dextroposition, underdevelopment of right pulmonary artery and anomalous systemic arterial supply from the descending aorta to the hypoplastic lung. A 36 year old female came with history of swelling in the right breast which turned up to be right breast fibroadenoma. Routine chest radiograph revealed scimitar syndrome which was confirmed on CECT chest.     

Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans

Isolated anomalous drainage of the left superior pulmonary vein into the left brachiocephalic vein was shown on computed tomographic (CT) scans. The CT findings include a vertical vein lateral to the aorticopulmonary window and aortic arch that terminates in the left brachiocephalic vein. Features that differentiate this entity from left superior vena cava, anomalous left brachiocephalic vein, and superior intercostal vein are discussed.     

24例镰刀综合征心血管造影征象分析

笔者分析了２４例镰刀综合征心肺血管病变的造影征象及其病理生理意义，并与手术（１４例）和尸解（４例）资料对照。２４例心血管造影示全部或部分右肺静脉异常引流，分别为１７例和７例。异常引流静脉近端局限性狭窄９例，其中２例异常和正常引流静脉间有侧支吻合，右下肺异常体动脉侧支供血１８例。并存各种心脏病变者１８例，马蹄肺６例，右下肺肺隔离症３例，下腔静脉闭锁和狭窄分别为２和１例。右下肺异常体动脉侧支和心脏病变是患者严重症状和肺动脉高压的主要因素。除肺隔离症外，经导管栓塞异常体动脉侧支可使用者明显受益。异常引流静脉狭窄具有限制血液分流的作用。     

Normative analysis of pulmonary vein drainage patterns on multidetector CT with measurements of pulmonary vein ostial diameter and distance to first bifurcation

RATIONALE AND OBJECTIVES: To document the frequency of normal and anomalous drainage patterns of the pulmonary veins, and to establish normal values for pulmonary vein ostial diameters, and distance to first bifurcation using multidetector computed tomography, as pertinent to ablation procedures for atrial fibrillation. MATERIALS AND METHODS: Two cardiothoracic radiologists retrospectively reviewed thin-section contrast material-enhanced multidetector computed tomography examinations of the thorax 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. Pulmonary vein anatomy was based on both the number of venous ostia and the drainage patterns of pulmonary veins. Pulmonary vein ostial diameters and distance to first bifurcation of the four major pulmonary veins (right inferior and superior, left inferior and superior) and any additional pulmonary veins were measured at a workstation using both axial images and multiplanar reconstructions by two experienced cardiothoracic radiologists; the mean pulmonary vein diameter and the shortest distance to first bifurcation of the two measurements are reported. RESULTS: The majority of patients, 82% (164 patients) had four pulmonary veins, with a superior and inferior ostium on the right and a superior and inferior ostium on the left. Of the remainder, 9% (18 patients) had five veins, 4.5% (9 patients) had three veins, 3% (6 patients) had two anomalies each, and 0.5% (1 patient) had three anomalies. The middle lobe pulmonary vein drained into the right superior pulmonary vein in 83.5% of patients, directly into the left atrium in 11% of patients, and into the right inferior pulmonary vein in 5.5% of patients; 6.5% of patients had a single left pulmonary vein ostium. Mean pulmonary vein diameters with 95% confidence intervals at the ostia were as follows: right superior 17.6 (13.64-15.36) mm; left superior 16.6 (16.03-17.08) mm; right inferior 17.1 (16.58-17.55) mm; left inferior 14.8 (14.25-15.27) mm, and independent middle lobe 8.6 (8.27-8.86) mm. Mean distance to first bifurcation with 95% confidence intervals were: right superior 14.5 (17.02-18.23) mm; left superior 17.6 (16.63-18.53) mm; right inferior 7.0 (6.49-7.46) mm; left inferior 13.5 (12.83-14.16) mm, and independent middle lobe 8.4 (7.7-9.17) mm. CONCLUSION: Thin-section thoracic computed tomography demonstrates a greater variability of pulmonary venous drainage than previously described. There is greater variability of the right lung venous drainage compared to the left lung. Eighty-two percent of people have four standard pulmonary veins. There is significant variability in pulmonary vein diameter and distance to first bifurcation.     

Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI)

Scimitar syndrome is a rare congenital pulmonary anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava or the right atrium. Very few reports are available that analyze the value of magnetic resonance imaging (MRI) in establishing the diagnosis. We present a case with Scimitar syndrome in which anomalous pulmonary venous return was confirmed by cine MRI.     

Transhepatic portosystemic collateral pathway via an accessory left gastric vein

An unusual transhepatic collateral was found in a patient with portal hypertension. This pathway originated from an anomalous portal branch that arose from the left portal vein branch, and was considered a venous analogy to an accompanying accessory left gastric artery. A relation between the development of the intrahepatic pathway and variation in the hepatic artery is suggested.     

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.     

Total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC), where the pulmonary venous circulation drains into the systemic venous circulation rather than into the left atrium, may present a number of problems at autopsy. A 5-week-old infant is reported who died suddenly and unexpectedly who was found at autopsy to have infradiaphragmatic TAPVC into the portal vein. The only associated anomalies were a tri-lobed left lung, a patent ductus arteriosus, and drainage of the inferior vena cava into the both the right and left atria. This case demonstrates particular features that may lead to difficulties in establishing the diagnosis of TAPVC. Sudden death in the absence of symptoms in this age group raises the possibilities of sudden infant death syndrome (SIDS) and inflicted injury, both of which were discounted in this case. An autopsy approach is outlined to maximize chances of diagnosing this entity that includes meticulous inspection and dissection of cardiac vascular connections before evisceration, with photographic documentation of findings. Concurrent cardiovascular abnormalities, including valve atresias, septal defects, and left heart hypoplasia are likely in the presence of asplenia/polysplenia syndrome. Extracardiac anomalies may be present in the form of Holt-Oram, Ellis-van Creveld, and cat eye syndromes. Careful examination of the common draining vein for obstructive features is an important part of the postmortem assessment. The possibility of heritable cases requires referral of the family for genetic evaluation.     

舌静脉引流的研究

目的:为中医舌诊和外科舌瓣提供舌的静脉引流规律.方法:用32具成年尸体进行观测,其中有7具为新鲜尸体的静脉腐蚀铸型标本.结果:舌的静脉引流,按管径及引流范围的大小,依次为舌下神经伴行静脉、会厌谷静脉、舌神经伴行静脉、舌根静脉和舌动脉伴行静脉.结论:中医舌诊所见的舌脉基础为舌下神经伴行静脉及舌神经伴行静脉;舌瓣的蒂部必须保留一个较通畅的静脉引流渠道.     

Total anomalous pulmonary venous connection to the portal vein

Anomalous pulmonary venous return represents a rare congenital anomaly with wide anatomic and physiologic variability. We report a case of a newborn with a rare form of total infracardiac anomalous pulmonary venous connection (TAPVC). The pulmonary veins draining both lungs formed two vertical veins, which joined to a common pulmonary trunk below the diaphragm. This venous channel connected to the portal vein through the esophageal hiatus. The diagnosis was suggested by color Doppler sonography and confirmed by intravenous digital subtraction angiography, which allowed definition of the anatomy.