Drainage of the inferior vena cava to the left atrium

Drainage of the inferior vena cava to the left atrium is an extremely unusual congenital heart disease. We describe a 54-year-old woman, in whom the diagnosis was suggested by transthoracic echocardiography, and then confirmed by a transesophageal exam and magnetic resonance imaging, which also revealed an associated secundum atrial septal defect. Surgical management involved reconstruction of the interatrial septum to include the inferior vena cava in the right atrium. The few previously reported cases in the literature are reviewed.     

Ostium primum atrial septal defect with persistent left superior vena cava opening into unroofed coronary sinus—A rare entity

Raghib syndrome is a rare developmental complex consisting of termination of the left superior vena cava in the left atrium, absence of the coronary sinus, and an atrial septal defect commonly located at the posterior‐inferior angle of the atrial septum. This complex was considered unique to Raghib syndrome; however, cases with a normal atrial septum have been reported where the orifice of the unroofed coronary sinus functions as the inter‐atrial communication. Our patient demonstrated an isolated persistent left superior vena cava draining into the left atrium through unroofed coronary sinus and presence of ostium primum atrial septal defect.     

Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Sinus venosus atrial septal defect (SV‐ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV‐ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio‐CT was finally conclusive. This diagnostic approach allowed the surgical planning.     

Echocardiographic aspects of persistence of the right valve of the venous sinus

The aim of this work is to demonstrate the possibility to identify persistent right sinus venous valve and to differentiate it from other right sided heart pathology by two dimensional echocardiography. We report the echocardiographic findings observed in three out of 215 paediatric patients we examined for clinically suspected congenital heart disease in 12 months period. The first patient was a 24 hours old newborn with transient pulmonary hypertension; the second one was a 6 months old child with pulmonary atresia, severe right ventricular hypertrophy, atrial septal defect and patent ductus arteriosus; the third patient, aged 6 months, had atrial septal defect and mild pulmonary stenosis. In all three patients a particular linear structure was seen, as a membrane that crossed the right atrium from the orifice of the inferior vena cava towards the atrial septum. This structure was identified as persistent right sinus venosus valve according to its morphology, its position into the right atrium and its connection to the atrial septum. The incidence of this echocardiographic finding was 1.4% in our series. In the first case contrast echocardiography from the inferior vena cava demonstrated the deviation of blood flow caused by the persistent sinus valve, although no intra-atrial pressure gradient was shown at cardiac catheterization. In the second patient who died during the operative procedure for making a systemic-pulmonary anastomosis, post mortem examination showed a membranous structure connecting the orifice of the inferior vena cava to the atrial septum.(ABSTRACT TRUNCATED AT 250 WORDS)     

Identification of atrial septal defects by cross-sectional contrast echocardiography.

Cross-sectional echocardiography, combined with injections of contrast into peripheral arm veins, has been used to study 15 patients with atrial septal defects and 10 patients with an intact interatrial septum. Of 11 patients with ostium secundum or sinus venosus atrial septal defects and left-to-right shunts a defect could be visualised in all, and in eight some degree of transfer of contrast from right atrium to left atrium was seen. In three of four patients with a dominant right-to-left shunt a defect was seen and in all there was free transfer of contrast from right atrium to left atrium. Though there may be variable loss of echoes in the septal image in patients with an intact interatrial septum, in general no fixed defect is seen an there is no transfer of contrast from right atrium to left atrium. This is a potentially valuable technique in the assessment of patients in whom an atrial septal defect is suspected.     

Transthoracic three-dimensional echocardiography prior to closure of atrial septal defects in children

AIMS: Our aims were to use transthoracic three-dimensional echocardiography to assess the morphology of atrial septal defects in children prior to closure, and to compare the three-dimensional echocardiographic data with transcatheter and surgical findings. METHODS AND RESULTS: We used transthoracic three-dimensional echocardiography in 62 consecutive patients, aged from 2 to 18 years, with atrial septal defects, measuring the maximal diameter and the extent of the rims. Subsequent to the study, we referred 42 patients for transcatheter closure, the rims being measured at greater than 4 mm. We found a good correlation between the maximal diameter of the defect as measured at transthoracic three-dimensional echocardiography and using a balloon (y = 3.45 - 0.73x; r = 0.78; p < 0.0001), the mean difference between the measurements being 2.4 +/- 2.8 mm. Successful closure with the Amplatzer septal occluder, having a mean size of 22 +/- 4 mm, was achieved in 95% of the patients. Of the original cohort, 20 patients were referred for surgical closure. In these patients, the inferior rim had been deemed insufficient in 5, the postero-superior rim in 6, and the postero-inferior rim in 9. Complete agreement was found when the deficiency of the rim as judged using transthoracic three-dimensional echocardiography was compared with intraoperative findings. The correlation between measurements of the deficiency of the rim achieved by transthoracic three-dimensional echocardiography and at surgery was excellent (y = 0.2 + 0.98x; r = 0.93; p < 0.0001), the mean difference between the measurements being no more than 0.6 +/- 0.4 mm. CONCLUSIONS: Transthoracic three-dimensional echocardiography proved accurate in measuring the maximal diameter and rims of atrial septal defects within the oval fossa. This non-invasive method will be valuable in selecting children for transcatheter or surgical closure of such defects.     

Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect

Background: Partial anomalous pulmonary venous connection (PAPVC) is frequently associated with atrial septal defect (ASD), especially sinus venosus defect (SVD). Although Waggstaffe described the pathology of SVDs in 1868, the exact anatomic features and the nature of SVD remains controversial. SVDs with no posterior atrial rim were observed in recent years. However, no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD. The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD. Methods: From January 2011 to December 2019, 256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled. Comprehensive review of preoperative transthoracic echocardiography, computed-tomography images and surgical findings were performed by experienced pediatric cardiologists. The subtypes of PAPVC, locations and types of ASD, and presence of posterior atrial rim of associated ASD were investigated. Results: PAPVC was right-sided in 244 children, left-sided in 6 children, and bilateral in 6 children. In PAPVC cases, ASD without posterior atrial rim existed in 226 SVD cases. ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium (RA) or to RA-superior vena cava junction. In cases with isolated ASD, there were 3 SVD, and the other 875 cases were secundum ASD. Conclusions: ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava (SVC) junction. For SVD, the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim, rather than adjacent to the SVC or to the inferior vena cava.     

Diagnostic echocardiographic features of the sinus venosus defect

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.     

Diagnostic echocardiographic features of the sinus venosus defect.

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.     

Subcostal real-time three-dimensional echocardiography of interatrial communications: reconstruction of an oval fossa defect, a superior sinus venosus defect with partially anomalous pulmonary venous drainage, an infero-posterior oval fossa defect, and a coronary sinus defect

Real-time three-dimensional echocardiography can surpass simple cross-sectional echocardiography in providing precise details of cardiac lesions. For the purpose of optimising treatment, we describe our findings with real-time three-dimensional echocardiography when interrogating different types of communications permitting interatrial shunting. A three-dimensional reconstruction of defects within the oval fossa enabled reliable identification of location, size, and integrity of surrounding rims. In the superior sinus venosus defect associated with partially anomalous pulmonary venous drainage, three-dimensional reconstruction helped to provide a better understanding of the relationship between the interatrial communication, the orifice of the superior caval vein, and the connections of the right upper pulmonary vein. In the defect opening infero-posteriorly within the oval fossa, three-dimensional reconstruction helped to avoid the risk of potentially inappropriate closure of the defect by suturing the hyperplastic Eustachian valve to the atrial wall, which could have diverted the inferior caval venous return into the left atrium, or obstructed the caval venous orifice. In the coronary sinus defect, three-dimensional echocardiography provided a 'face to face' view of the entire coronary sinus roof, showing a circular defect communicating with the cavity of the left atrium. Acquisition of the full-volume data sets took less than 2 minutes for the patients having defects within the oval fossa, and no more than 3 minutes for the patients with the sinus venosus and coronary sinus defects. Post-processing for the defects in the oval fossa took from 5 to 8 minutes, and from 12 to 16 minutes for the more complicated defects. CONCLUSION: Cross-sectional two-dimensional echocardiography can establish correct diagnosis in all types of atrial communications; however, real-time three-dimensional reconstruction provides additional value to the surgeon and interventionist for better understanding of spatial intracardiac morphology.     

Detection of sinus venosus atrial septal defect by two-dimensional echocardiography.

In a 4-year period, 10 patients (mean age, 3.6 years) with a superior type sinus venosus atrial septal defect were investigated by two-dimensional echocardiography. The defects were easily visualized in all patients using a long axis vena cava superior-inferior plane with a subcostal approach, while the atrial septum seemed to be intact in the coronal plane conventionally used for atrial septal defects of the primum or secundum type. Additional colour flow mapping, performed in two patients, demonstrated shunting across the depicted defect. In our experience, the modified plane is of great diagnostic value in cases with a sinus venosus defect.     

Transoesophageal echocardiographic assessment of primum, secundum and sinus venosus atrial septal defects

We compared the ability of transthoracic and transoesophageal echocardiography to determine the presence and site of an atrial septal defect and associated anomalous pulmonary venous connexions in 13 school age children (aged 5 to 15 years) and 12 adults (aged 25 to 68 years). Transthoracic echocardiography detected atrial septal defects in 12 children and 6 adults. Transoesophageal echocardiography confirmed the position of 16 (13 secundum, 3 primum) of these 18 defects but altered the diagnosis from a secundum defect to a sinus venosus defect in one and from a sinus venosus defect to a high secundum defect in another. In addition to these 18, transoesophageal echocardiography diagnosed a defect in 5 adults (3 secundum and 2 sinus venosus defects) and 1 child (secundum defect). In an adult with inconclusive transthoracic findings, transoesophageal echocardiography enabled clear visualisation of the atrial septum and excluded an atrial septal defect. Transoesophageal echocardiography showed anomalous attachment of a pulmonary vein into the region of a sinus venosus defect (n = 3) but did not show anomalous connexions to the superior caval vein (n = 3) or the inferior caval vein (n = 1). Transoesophageal echocardiography provides a reliable method of diagnosing or excluding an atrial septal defect in patients with inconclusive transthoracic findings and is of particular diagnostic value in sinus venosus defects.     

Assessment of muscular ventricular septal defect closure by transcatheter or surgical approach: a three-dimensional echocardiographic study.

AIMS: Previous classification of muscular ventricular septal defects (VSDs) visualized on two-dimensional echocardiography relied on artificial divisions of the septum. New visualization of the ventricular septum integrating the third dimension would facilitate communication between cardiologists and surgeons. The objectives of this study were (1) to assess in patients with muscular ventricular septal defects the accuracy of left ventricular three-dimensional echocardiographic reconstructions in demonstrating the position, the size and the tissue rims of the defects; (2) to compare findings by three-dimensional echocardiography with those obtained by surgical and transcatheter approaches. METHODS AND RESULTS: Twenty-six patients, aged from one month to 40 years, with muscular ventricular septal defects underwent three-dimensional echocardiographic study. From the left ventricular three-dimensional echocardiographic reconstructions, the localization, the maximal diameter and the tissue rim of the defect were analysed and compared with surgical or transcatheter findings. Optimal three-dimensional echocardiographic reconstructions were obtained in 22 patients. Nineteen had a single muscular ventricular septal defect and three had multiple muscular ventricular septal defects. The muscular ventricular septal defect localizations were the inlet septum in three, the outlet septum in three, the mid-muscular septum in 14 and the apex in eighth. In 10 patients who underwent surgical closure, the correlation between three-dimensional echocardiography and surgery for muscular ventricular septal defect maximal diameter was y=0 x 95 x +0.13 (r=0.98; P<0.001). The agreement between three-dimensional echocardiographic and intraoperative findings on muscular ventricular septal defect localization were complete. In five patients who underwent transcatheter closure, the mean difference between three-dimensional echocardiographic maximal diameter and stretched diameter was 1 x 8+/-0 x 5 mm. CONCLUSION: The three-dimensional echocardiographic left ventricular views provide a new and easily communicated visualization of various muscular ventricular septal defects. Such new imaging should contribute to the surgical and transcatheter treatments of muscular ventricular septal defects.     

3D-transthoracic echocardiography: a selection method prior to percutaneous closure of atrial septal defects

Prior to putting in place a percutaneous device, the assessment of the atrial septal defect anatomy is mandatory. The 3D transthoracic echocardiography is a non-invasive method bringing an imaging of the septal defect surface and its borders. Fifty-two patients ageing from 3 to 16 years old had a rotational 3D transthoracic echocardiography. Thirty-six (69%) were selected for a percutaneous closure (borders > 7 mm and a septal surface/atrial septal defect ratio > 2). Thirty-two of these selected patients (89%) benefited from the Amplatz prosthesis implantation with success. The maximal 3D diameter of the septal defect was 20 + 4 mm (14-30) compared to the mean size of prosthesis at 22 mm (18-30). Four of the 36 patients (aneurismal septum or a borderline septal surface/atrial septal defect ratio) were secondarily oriented to surgeons. Sixteen patients (31%) were selected directly to a surgical closure. The lack of borders or septal surface were confirmed by the surgical view. The 3D transthoracic echocardiography allows to define new criteria for the selection of patients prior to a percutaneous closure of atrial septal defects. This prospective study demonstrates that 9 out of 10 patients who had the 3D criteria had a percutaneous closure of the septal defect.     

Echocardiographic considerations during deployment of the Helex Septal Occluder for closure of atrial septal defects

The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.     

Diagnosis of atrial septal defect using magnetic resonance imaging

We studied the morphological features of defects of the interatrial septum using magnetic resonance imaging (MRI) to determine the sizes of defects and other abnormalities. MR images were obtained in 28 patients with atrial septal defect, including five cases with complicated anomalies (two with Ebstein's anomaly, one pentalogy of Fallot, and one anomalous pulmonary vein connection and azygos continuation). Images were also obtained in the control subjects including seven normal volunteers and 142 patients with various acquired heart diseases. The diagnosis of atrial septal defect was established by cardiac catheterization, angiography and two-dimensional echocardiography prior to the MRI studies, and in 14 patients, the diagnosis was confirmed by surgery. The MRI unit had a superconducting magnet and operated at 0.25 or 0.50 Tesla. A spin echo pulse sequence was used with an echo time of 40 or 60 msec. At the beginning of this study, non-gated MRI images were obtained in the 28 controls and in three patients with atrial septal defect. Nongated MRI could not image the anatomical structure of the interatrial septa of 12 of the 28 controls, or any of the three patients with atrial septal defect. Nongated MRI was, therefore, inadequate for visualizing cardiac anatomy. Gated MRI images were obtained in 141 controls and in 25 patients with atrial septal defect. Gated MRI revealed the interatrial septum, interventricular septum, atrioventricular septum, mitral valve, tricuspid valve and other intracardiac structures in most subjects. In 17 control subjects (12%), however, there was a very faint signal from the central portion of the interatrial septum. In these instances, there was a gradual fading of the signal of the interatrial septum, so that they could be distinguished from the atrial septal defect. The sudden disappearance of the signal from the interatrial septum was observed by gated MRI in all 25 patients with atrial septal defect. The sizes of the defects by MRI coincided with the findings at surgery in all 14 patients. MRI showed right atrial dilatation, right ventricular hypertrophy and dilatation, and pulmonary artery dilatation in most of the patients having atrial septal defect. Complex anomalies associated with atrial septal defect were also clearly shown by MRI, such as displacement of the tricuspid leaflets in two patients with Ebstein's anomaly, and anomalous pulmonary venous connection and persistent left superior vena cava in one patient. These results indicated that gated MRI is a valuable noninvasive method of diagnosing atrial septal defect and complicating anomalies.     

Transthoracic real-time three-dimensional echocardiography using a fan-like scanning approach for data acquisition: methods, strengths, problems, and initial clinical experience

Three-dimensional echocardiography is an emerging clinical method to assess cardiovascular disorders. The feasibility of using a linear mode scanning (parallel slicing) for transthoracic data acquisition has been demonstrated. In this study, we evaluated the feasibility of real-time transthoracic three-dimensional imaging of the heart using a fan-like scanning mode of echocardiographic data acquisition. We used a computer-driven motor to sequentially angulate transthoracic transducers over a fan-like arc up to 90 degrees. With careful ECG and respiratory gating, we acquired basic two-dimensional data set via parasternal and subcostal windows and performed dynamic three-dimensional reconstructions. The problems encountered included the need to repeat data acquisition sequences because of transducer movement or inappropriate gain and gray scale settings. From 15 scanning sequences in four patients, we were able to use ten sets of data. These yielded good quality three-dimensional studies projecting normal valves, a stenotic mitral valve, and an atrial septal defect, in a number of novel views. The valves could be visualized from above and from below as well as in other orientations, and the detailed anatomy appraised. Spatial relationships of the atrial septal defect with inferior and superior vena cava, coronary sinus, or tricuspid annulus could be uniquely displayed through views from the right side of the heart. This technique provided adequate new imaging planes not available from two-dimensional echocardiography. This experience demonstrates for the first time that transthoracic three-dimensional echocardiography using a fan-like scanning mode of data acquisition is feasible, and that it provides adequate visualization of intracardiac structures in unique projections.(ABSTRACT TRUNCATED AT 250 WORDS)     

Detection of ostium secundum atrial septal defects by transoesophageal cross-sectional echocardiography

Transoesophageal cross-sectional echocardiography has special advantages when investigating the interatrial septum which is imaged perpendicularly without echo dropouts from an oesophageal transducer position. The technique was successfully used in 19 out of 20 patients (95%) with an ostium secundum atrial septal defect and in 30 control subjects. In all of the latter the interatrial septum was visualised as a continuous echo structure separating the atria, whereas a distinct discontinuity representing the septal defect was apparent in all patients with atrial septal defect. Echocardiographic measurement of the defect size correlated well with surgical findings in 11 patients who underwent open heart surgery in the course of this study. In a comparative transthoracic examination, adequate recordings were obtained in 18 of the 20 patients and in 26 of the 30 control subjects. Direct subcostal visualisation of the defect was reliable in 10 of 18 patients. Peripheral venous contrast studies were also performed with the transoesophageal as well as the transthoracic technique. Echo contrast remained confined to the right heart in the control subjects. Left sided contrast appearance diagnostic of an interatrial communication was shown in the patients using the transoesophageal technique (100% sensitivity), with an additional right atrial negative contrast apparent in seven patients. The transthoracic approach, on the other hand, showed left sided echo contrast in 14 of 18 patients and an additional negative contrast effect in two of the 14. It is concluded that transoesophageal is superior to transthoracic cross-sectional echocardiography as a highly sensitive method for the detection and evaluation of ostium secundum atrial septal defects.     

Echocardiographic features of total anomalous pulmonary venous drainage into the coronary sinus

Echocardiograms were obtained from five infants with total anomalous pulmonary venous drainage to the coronary sinus or the portal system or the superior vena cava and from one child with a secundum atrial septal defect and a large coronary sinus due to persistence of the left superior vena cava. The results demonstrate that an enlarged coronary sinus is positioned consistently posterior to the left atrium in approximately the same horizontal plane as the aortic valve. Echocardiographically the coronary sinus can be located as an echo complex behind the left atrium by using the aortic root as a reference point. The echo complex can be differentiated from the other spurious echoes in the left atrium by its characteristic phasic motions. The additional demonstration of the enlarged common pulmonary venous chamber behind the right atrium confirms the diagnosis of anomalous pulmonary venous drainage to the coronary sinus. For other types of anomalous pulmonary venous return, anatomic diagnosis with single crystal M mode echocardiography is not always possible because of the positional variability of the common pulmonary venous chamber in relation to the left atrium.     

Cor Triatriatum Dexter versus Prominent Eustachian Valve in an Adult Congenital Heart Disease Patient

An eustachian valve (EV) remnant, if present, is usually noted by the presence of a thin ridge or a crescent‐shaped fold of endocardium arising from the anterior rim of the inferior vena cava orifice due to the persistence of the right sinus venosus valve. Though the embryologic explanation of cor triatriatum dexter (CTD) is the same as that of the normal formation of the EV—lack of regression of the right sinus venosus valve—it is usually called CTD or divided right atrium when there are attachments on the atrial septum giving the appearance of a divided atrium. However, it's called prominent eustachian valve when the right sinus venosus valve has partly regressed, with no remaining septal attachments and without the appearance of a divided atrium. We present the case of an adult patient with an atrial septal defect with a high insertion of a giant EV, which mimics the echocardiographic appearance of divided right atrium.