Partial anomalous pulmonary venous return with secundum atrial septal defect: A case report

Partial anomalous pulmonary venous return (PAPVR) is a congenital heart anomaly in which some of the pulmonary veins return to the right atrium or one of its supplying veins instead of normally connecting with the left atrium. Oftentimes it is concurrent with a secundum atrial septal defect. PAPVR is typically asymptomatic, however symptoms of pulmonary hypertension can arise at higher degrees of left-to-right shunting. An 80-year-old male presented with exertional dyspnea and was found to have a secundum atrial septal defect on echocardiogram. A subsequent contrast enhanced computed tomography of the chest revealed a concomitant PAPVR.     

Partial anomalous pulmonary venous return in Turner syndrome

PurposeThe aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome.Methods and resultsAll Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients.ConclusionWhen studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients.     

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.     

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.     

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。     

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.     

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.     

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.     

Role of multi-slice CT angiography in the evaluation of pulmonary venous anomalies

Purpose and aim

To assess the role of MSCT angiography in evaluation of extra-cardiac vascular abnormalities in pulmonary venous anomalies and compare it with echocardiography.

Materials and methods

This study included 26 patients with an age range of 15 days–25 years. All of these patients underwent MSCT angiography and echocardiography. Only 10 patients underwent cardiac catheterization.

Results

Our initial experience showed that MSCT is capable of complementing echocardiography and replacing diagnostic cardiac catheterization for anatomical delineation if performed with an optimum technique.MSCT angiography proved to be a worthy primary investigation tool in patients whom ECHO has been able to clearly identify the intracardiac anatomy, but not the extra-cardiac vascular anatomy.

Conclusion

MDCT correctly depicted the TAPVR (Total anomalous pulmonary venous return) and PAPVR (Partial anomalous pulmonary venous return) types of pulmonary venous anomalies with sensitivity 100%, and specificity 100%. The specificity of echocardiography was 50% for both findings. Inspite of the risk of ionizing radiation and contrast medium injection the adoption of our minimal invasive, low radiation, non-ECG gated protocol greatly reduces the time, radiation dose, and contrast medium volume needed to perform an optimum CT angiographic technique. Thus, paving a clear road map for pre and post operative assessment of patients with pulmonary venous anomalies.     

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.     

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.     

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.     

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.     

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.     

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 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.     

Catheter-Malposition-Induced Cardiac Tamponade via Contrast Media Leakage During Computed Tomography Study

We present a rare case of a central venous catheter-malposition-induced life-threatening cardiac tamponade as a result of computed tomography (CT) with contrast enhancement in an infant with a ventricular septal defect and pulmonary atresia after a modified Blalock–Taussig shunt. The diagnosis was confirmed by chest radiographs and CT study with catheter perforation through the right atrial wall and extravasation of the contrast medium into the pericardium, leading to cardiac tamponade and subsequent circulatory collapse. Two hours after successful cardiopulmonary resuscitation, the patient gradually resumed normal hemodynamic status.     

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。