CT and MR imaging of partial anomalous pulmonary venous return to the azygos vein

Partial anomalous pulmonary venous return to the azygos vein is very unusual, occurring in 0.02% of autopsy examinations. We report the CT, magnetic resonance, and angiographic findings in a 67-year-old man with partial anomalous venous return to the azygos vein.     

Computed tomography diagnosis of partial anomalous pulmonary venous drainage

A case of the rarely occurring partial anomalous pulmonary venous drainage of the right upper lobe into the azygos arch is presented. Computed tomography (CT) clearly demonstrated the abnormal vessel coursing through the right upper lobe and draining into the azygos arch.     

Dual connection of single pulmonary vein in partial anomalous pulmonary venous return

We present the case of a 6-month-old infant born premature at 29 weeks with perinatal stroke and postnatal hypoxia. Echocardiogram was suspicious for partial anomalous pulmonary venous return (PAPVR). Cardiac CT showed an unusual variant of PAPVR, with a vertical vein having a dual connection superiorly to the left innominate vein and inferiorly to the morphologic left atrium. This unusual variant has the potential for right-to-left flow with a possibility of systemic hypoxia and paradoxical embolism.     

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.     

Tc-99m MAA findings in dilated cardiomyopathy with partial anomalous venous connections

Tc-99m MAA showed asymmetric uptake in the lung field in a 21-year-old man with dilated cardiomyopathy. CT revealed partial anomalous venous connections in the left upper lobe. Angiogram of the left pulmonary upper lobe showed all the contrast material drained into the left vertical vein. The possible cause of relative increase in the left upper lobe blood flow is that right pulmonary blood flow is slowed by the high pressure in the left atrium due to dilated cardiomyopathy, whereas the flow from the left upper lobe drains into the superior vena cava which has less pressure than left atrium.     

MR diagnosis of subdiaphragmatic anomalous pulmonary venous drainage in a newborn

Our report describes a case of infradiaphragmatic total anomalous pulmonary venous return diagnosed by MR in a newborn with an interruption of the aortic arch with ventricular septum defect and anomalous pulmonary venous drainage. The severity of the congenital cardiopathy did not permit surgical treatment and the infant died soon after. Pathology confirmed the MR findings. Magnetic resonance not only complements echocardiography but also can be used for patients in intensive care and can in our case avoid angiography.     

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。     

MRI of partial anomalous pulmonary venous return (scimitar syndrome)

We report a case of anomalous pulmonary venous drainage into the inferior vena cava (scimitar syndrome). Cine MRI and 3-D contrast-enhanced MR angiography provides an non-invasive diagnostic technique in the evaluation of anomalous pulmonary venous return.     

The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return

An anomalous pulmonary vein draining into the subdiaphragmatic inferior vena cava was initially demonstrated on computed tomographic (CT) scans. The diagnosis of scimitar syndrome was confirmed with digital subtraction angiography. In retrospect, the anomalous vein and dextroposition of the heart were shown on chest radiographs.     

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.     

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.     

Turner syndrome with aberrant right subclavian artery and partial anomalous pulmonary venous return

We report the case of a 6 year old girl with Turner syndrome and aberrant right subclavian artery with an incidental finding of PAPVR on contrast-enhanced, high-pitch, 128-slice, electrocardiographic-gated dual source CT. There is value of using high-pitch DSCT in pediatric patients for diagnostic images with minimal radiation exposure.     

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.     

应用超声CDFI诊断完全性肺静脉异位引流

目的：探讨应用超声CDFI在诊断小儿完全性肺静脉异位引（TAPVC）价值。方法：应用超声CDFI诊断的37例小儿TAPVC与手术结果进行对比分析。结果：37例患者中心内型肺静脉异位引流18例,心上型12例,心下型7例。超声CDFI对小儿TAPVC诊断的正确率100％。结论：应用超声CDFI可心正确地诊断小儿TAPVC。     

Single-breath-hold venous or arterial flow-suppressed pulmonary vascular MR imaging with phased-array coils

A method for acquiring pulmonary vascular magnetic resonance (MR) images with either venous or arterial flow suppression is described. The proposed method only marginally increases the overall imaging time compared with conventional flow-suppression techniques. This enables an acquisition to be completed within a single breath hold with some selectivity as to flow direction. Instead of applying a spatially selective presaturation pulse before each radio-frequency (RF) excitation pulse, the flow presaturation pulse is applied once every 16-20 RF excitation pulses. To avoid image artifacts and to maintain a steady state, each presaturation pulse interval is followed by a normal imaging segment but with data acquisition turned off. Overall imaging time is increased by two TR intervals for each presaturation segment. For a 256 × 128 matrix acquisition, venous flow presaturation increases overall imaging time by approximately 14 TR intervals, while arterial flow suppression increases imaging time by 10 TR intervals.     

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.     

完全性肺静脉异位引流的外科矫治

目的总结完全性肺静脉异位引流(TAPVR)的外科治疗经验,以提高手术疗效。方法 22例患者中男10例,女12例。年龄2～14岁。心上型8例,心内型13例,混合型1例。全组均在中度低温体外循环下行TAPVR矫治术。结果手术死亡1例,死于术后严重低心排出量综合征;术后并发心律失常3例,呼吸道感染2例。结论完全性肺静脉畸形引流一经确诊应尽早手术。通过手术技术的改进心上法治疗心上型TAPVR,手术部位显露好,术后心律失常发生率低。TAPVR的外科矫治可取得满意的疗效。