Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging,pitfalls, and caveats

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Right Superior Vena Cava Connected Left Atrium with Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum: An Unusual Cause of Paradoxical Embolism

A six-month-old girl presented with history of convulsion and cyanosis, was eventually found to have a right superior vena cava connected to left atrium with anomalous upper right pulmonary vein return and without atrial septal defect. Diagnosis was confirmed by cardiac magnetic resonance imaging. The patient underwent a successful cardiac surgery correction, routing SVC to right atrium and repair of right upper pulmonary vein to left atrium.     

Differential pulmonary flow in hypoplastic left heart syndrome.

We report a case of hypoplastic left heart syndrome associated with restrictive interatrial communication and partial anomalous pulmonary venous connection via a right lower pulmonary vein draining to the inferior vena cava. We found unequal pulmonary artery pressure and different pulmonary artery structure, with the right pulmonary artery being lower in pressure and more tortuous and dilated in its peripheral branches than the left. This was attributed to the variant degrees of pulmonary venous obstruction. The left pulmonary venous return was severely obstructed by the restrictive interatrial communication, whereas the anomalous right lower pulmonary vein drained into the inferior vena cava, with less obstruction. To the best of our knowledge, there has never before been a report of differential pulmonary flow associated with a partial anomalous pulmonary vein, in a case of hypoplastic left heart syndrome.     

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment

Background and purposeSinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium.Summary of caseWe report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect.ConclusionsInterventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.     

Masked abnormal drainage of the inferior vena cava into the left atrium

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Successful transcatheter occlusion of an anomalous pulmonary vein with dual drainage to the left atrium

We describe a case of a scimitar syndrome “variant” where dual drainage existed from the right upper and middle pulmonary veins to the inferior vena cava and left atrium. Device closure of the anomalous vein at the level of the connection to the IVC was successful in achieving diversion of pulmonary venous flow to the left atrium. Vigilance during work‐up of anomalous pulmonary venous drainage (whether isolated or associated with other cardiac defects that may be amenable to device closure) is important to define the presence of dual connections to the left atrium, in which case a less‐invasive transcatheter approach may be feasible. © 2015 Wiley Periodicals, Inc.     

Partial anomalous pulmonary venous return showing anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disease with a reported incidence of autopsied case. The location of the anomalous pulmonary venous return is usually the right atrium, superior vena cava (SVC), and sometimes the brachiocephalic vein, inferior vena cava (IVC) or coronary venous sinus. Recently we experienced a rare case of PAPVR showing anomalous right total pulmonary venous return to the azygos vein. Furthermore, downward translocation of the right upper lobe bronchus was evident. This rare case is reported along with a review of the related literature.     

Single left pulmonary vein with normal pulmonary venous drainage: Association with partial anomalous pulmonary venous return

An unusual case of a single left pulmonary vein draining the left lung and joining the left atrium without venous obstruction is reported. This anomaly occurred in association with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because of the charácteristic radiologic appearance of this anomaly. It is important to distinguish this benign entity from more ominous pulmonary radiodensities.     

An unusual opacity of the right base

The scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable malformation of the right lung characterized by an abnormal right sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. We present a case in which this diagnosis was suspected on an abnormal routine chest radiograph in a 38-year-old asymptomatic woman. Most patients are asymptomatic; symptomatic patients have a marked left-to-right shunt or a severe congenital heart disease. They usually suffer from shortness of breath, asthenia or repeated chest infections. Usually, the posteroanterior chest radiograph can confirm the diagnostic. It shows the abnormal vein draining into the inferior vena cava as a curved vascular shadow with a scimitar like appearance. However, in some cases, when the scimitar vein is masked by the overlying cardiac shadow, computed tomography, angiography and magnetic resonance imaging can be helpful by showing the abnormal vein and its insertion into the inferior vena cava. Scimitar syndrome seldom necessitates surgical intervention. However, repeated lung infections can sometimes require lobectomy or pneumonectomy, left-to-right shunt vascular surgery to redirect the scimitar vein into the left atrium.     

Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium.

A 4-month-old infant with cyanosis but without other abnormal cardiac findings is presented in whom the diagnosis of anomalous systemic venous connection to the left atrium was made by contrast echocardiography. The diagnosis was later confirmed by cardiac catheterisation and selective cineangiography. When saline was injected into a vein on the dorsum of each hand while echocardiographically recording the cardiac structures, the left atrium, left ventricle, and aorta were opacified without visualisation of the right ventricle. Similar study with injection into the right foot produced opacification of the right ventricle without visualisation of the left-sided structures. These data suggested normal drainage of the inferior vena cava with anomalous connection of the superior vena cava to the left atrium. A review of the previously reported cases of anomalous connection of the right superior vena cava to the left atrium is presented together with the possible embryological origin of this anomaly.     

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as “the scimitar sign”, is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.     

Surgical case of partial anomalous pulmonary venous connection to superior vena cava with cor triatriatum: Williams’ modification and excision of diaphragm

We experienced a successful pediatric surgical case of partial anomalous pulmonary venous connection to the superior vena cava with cor triatriatum. Echocardiography and multidetector-row computed tomography showed partial anomalous pulmonary venous connection (right upper pulmonary vein connected to the high superior vena cava) and atypical cor triatriatum (analogue to type III-A2 of Lucas-Schmidt classification: left upper pulmonary vein had dual connection to the innominate vein via vertical vein and the accessory chamber). At 8 years of age, the male patient underwent extracardiac right atrial pedicle repair of partial anomalous pulmonary venous connection to the superior vena cava (Williams’ modification) and excision of the diaphragm between the accessory chamber and the left atrium simultaneously. The postoperative course was uneventful in normal sinus rhythm and there was no stenosis of newer drainage root from right upper pulmonary vein.     

Total anomalous pulmonary venous draining to inferior vena cava

A 12-year-old girl complained of tachycardia and dyspnea after exertion but without cyanosis. Her echocardiogram showed an ASD and a widened inferior vena cava. A difference in PO2 between inferior and superior vena cava was evident. An anastomosis between left atrium and pulmonary vein, and a long oval pericardium to cover the opening of the descending vein and ASD into left atrium were performed during cardiopulmonary bypass. The patient recovered without complications.     

Tight mitral stenosis with normal unilateral pulmonary capillary pressure

There are few reports of severe mitral stenosis with normal pulmonary artery wedge pressure. In order to illustrate this problem we present two adult patients with these abnormalities. Both patients had intact interatrial septum with partial anomalous pulmonary venous drainage, one to the inferior vena cava (The Scimitar Syndrome) and the other to the superior vena cava. In the first case the right pulmonary artery wedge pressure was 11 mm Hg. In the second case this pressure was only 8 mm Hg. Pulmonary arteriograms are of paramount importance in the diagnosis and surgical management. In both cases the surgical procedure with mitral commissurotomy and transplant of the anomalous pulmonary veins to the left atrium was successful.     

Intraatrial conduit repair in Scimitar syndrome

A 26-year-old woman with partial anomalous pulmonary venous drainage into the right atrium (Scimitar syndrome) was successfully operated upon by incorporating an intra-atrial conduit. The single right pulmonary vein present was connected to the right atrial inferior vena cava junction. The atrial septum was extremely deviated to the left, and the left atrium was located entirely on the left of the spine. A small patent foramen ovale was found. The distance between the orifice of the right pulmonary vein and the enlarged atrial septal defect was too great to use an intracardiac patch, so a 14 mm woven Dacron graft, 6 cm long, was interposed between them. The patient is doing well 21 months following the operation.     

Unusual Case of Left Partial Anomalous Pulmonary Venous Connection with Successful Surgical Management

We describe a case of left-sided partial anomalous pulmonary venous connection diagnosed in the setting of a respiratory illness affecting the right lung, which presumably elevated pulmonary pressures and worsened right-to-left shunting. The anatomical configuration appears to be highly unusual in this case, with separate supracardiac connections of the left upper pulmonary vein (LUPV) to a vertical vein (draining to the innominate vein) and the left lower pulmonary vein to the azygos vein. Successful surgical repair was undertaken with direct anastomosis of the LUPV to the left atrial appendage and creation of an intra-superior vena cava baffle to redirect azygos vein flow to the left atrium.     

Budd-Chiari syndrome due to membranous obstruction of the inferior vena cava of congenital origin. Ten-year follow-up after radiologic treatment]

We report a case of Budd-Chiari syndrome due to membranous obstruction of the inferior vena cava with a congenital malformation composed of a left inferior vena cava at the sub-renal level with an azygo-caval continuation without a retrohepatic segment of the inferior vena cava. To our knowledge, this is the first report of this association. The congenital venous malformation suggests a congenital etiology to the inferior vena cava membrane. The membranous obstruction was treated by percutaneous transluminal angioplasty. During the 10-year follow-up, there was no recurrence of the membranous obstruction as seen with Doppler ultrasound and magnetic resonance imaging.     

Correction of anomalous drainage of right superior vena cava to left atrium

A 2-year-old boy with cyanosis was found to have normal situs and looping with anomalous drainage of a right-sided superior vena cava to the left atrium, and intact interatrial septum in association with anomalous drainage of the left pulmonary veins to the right superior vena cava. He underwent successful surgical repair of this rare congenital malformation.