Inferior Sinus Venosus Defect Research Articles

Transcatheter correction of residual inferior sinus venosus defect after surgical closure.

Transcatheter correction of residual inferior sinus venosus defect after surgical closure.

Transcatheter closure of inferior sinus venosus defect using a patent ductus arteriosus occluder following simulation with a 3D-printed model.

BackgroundTranscatheter closure of inferior sinus venosus defect (ISVD) is still contraindication. To explore whether transcatheter closure with patent ductus arteriosus (PDA) occluders is possible for ISVD.MethodsFrom June 2014 to March 2021, 12 patients were recruited diagnosed as <25 mm ISVD. The three-dimensional printing (3DP) heart model was produced based on multi-slice computed tomography (MSCT) scans. Preoperative closure simulation was planned on the personalized 3D model for each patient. Follow-up including electrocardiography (ECG), transthoracic echocardiography (TTE), and X-ray was traced.Results3DP models of 12 patients were successfully printed. Twelve patients had been diagnosed with <25 mm ISVD and 4 of them had another secundum atrial septal defect (ASD). All patients were produced interventional therapy successfully. PDA occluder was implanted to closed ISVD, and ASD was closed using ASD occluder simultaneously. The average diameter of ISVD measured by TTE was (12.67±3.80), and the average diameter of sagittal axes and longitudinal axes measured by the 3D-printed model was (17.08±3.20) and (18.42±4.62) mm, respectively. The average size of PDA (diameter of pulmonary artery side) was (28.17±3.35) mm. Compared with the preoperative, the X-ray cardiothoracic ratio (0.51±0.04 vs. 0.47±0.06, P=0.007) and the right ventricle anterior-posterior diameter (31.17±5.65 vs. 24.58±3.75 mm, P<0.001) of postoperative was significantly decreased. During the average (47.75±27.52) months follow-up, it has achieved satisfying results, and there were no severe adverse events such as device transposition, death, and pericardial tamponade occurred.ConclusionsAssisting by 3D heart model, transcatheter closure of ISVD with PDA occluder had an excellent outcome. This method provides a new considerable treatment strategy for ISVD.

Inferior sinus venosus defect and anomalous hepatic venous return to the coronary sinus leading to an Eisenmenger syndrome.

Inferior sinus venosus defect associated with left hepatic vein drainage to the coronary sinus is an extremely rare condition. We report the case of a 41-year-old man suffering from pulmonary arterial hypertension related to this unusual CHD. Planning of heart-lung transplantation in this case required accurate anatomical description.

New Insights into Sinus Venosus Defects from Cross-Sectional Imaging

Sinus venosus defects include two varieties, superior and inferior sinus venosus defects. The superior sinus venosus defect is characterized by abnormal communication between two closely related venoatrial structures: 1) the normally positioned superior vena cava-right atrium complex and 2) the right pulmonary vein-left atrium complex that is displaced leftward, forward and upward. Inferior sinus venosus defects primarily involve the inferior vena cava-right atrial junction while the right pulmonary vein-left atrial junction can also be affected. Because of the rarity and wide variation of the defects, the morphological characterization of sinus venosus defects is inconsistent among investigators and often inaccurate. Modern imaging technologies with high spatial and temporal resolutions have allowed accurate and detailed assessment of the pathological anatomy in larger numbers of cases. In this pictorial essay, we revisit the established and controversial features of the sinus venous defects using two-dimensional (2D) and three-dimensional (3D) images obtained by magnetic resonance (MR) or computed tomography (CT) with brief discussion on imaging and treatment strategies.

Cross sectional imaging is transforming the diagnosis of rare congenital defects

Abstract Aim Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly in which at least one of the pulmonary veins do not drain into the left atrium. Patients with PAPVC can often be asymptomatic and the presence of right heart dilatation on echo should prompt assessment of pulmonary venous anatomy. The aim of this study was to compare the anatomical features of a contemporary cohort of patients with PAPVC with previous historical cohorts, in order to determine whether the use of 3 dimensional imaging techniques has altered the diagnostic yield of PAPVC anatomical subtypes. Methods A retrospective review of patients diagnosed with PAPVC on the basis of CT and/or MRI imaging, between 2010 and 2020. Results 45 cases of PAPVC were found. 60% were women with an average age of 53 years (17–85 years). 25 cases were diagnosed on MRI and 20 on CT, with CT scanning being used with increasing frequency during the period covered. The most common pattern was right-sided involvement, with isolated right PAPVC in 36 patients (80%) – see chart 1. Of these, the majority had isolated right upper PAPVC (19, 53%); 13 (36%) had right upper and middle PAPVC, 1 had isolated right middle PAPVC, 2 had isolated right lower PAPVC, and 1 had unilateral right-sided PAPVC. Bilateral involvement was seen in 4 patients (9%), isolated left-sided involvement in 3 (7%), and right upper and middle and left upper PAPVC in 2 (4%). Associated sinus venosus defects (SVDs) were only detected in 15 cases (33%): 14 had right-sided APVC and 1 had bilateral upper PAPVC. There were 13 superior SVDs, 1 inferior SVD and 1 coronary sinus SVD (see chart 2). 13 of the 27 (48%) women had Turner's Syndrome, Most had right-sided PAPVC (8, 61%), with bilateral involvement in 3 (23%) and left-sided PAPVC in 4 (31%) – see chart 3. 17 (38%) of the patients have had their PAPVC surgically redirected, of which 13 (29%) had an associated SVD. Conclusion With Increasing use of cross sectional imaging, we are seeing anatomical heterogeneity in patterns of PAPVC compared to historical cohorts1,2. Interatrial communications were less frequently seen, with unusual patterns of drainage seen more frequently in our Turner's Syndrome group. Funding Acknowledgement Type of funding sources: None. CT scan of bilateral PAPVC

B-PO05-217 SPONTANEOUS REGIONAL ISOLATION IN THE RIGHT ATRIUM WITH DISRUPTION OF INTRA-ATRIAL CONDUCTION MORE THAN A DECADE AFTER ATRIAL ABLATION AND SURGICAL CORRECTION OF SINUS VENOSUS DEFECT

Regional isolation of the right atrium (RIRA) can occasionally occur at the time of catheter ablation (CA) for atrial arrhythmias in congenital heart disease. We present a case of RIRA arising spontaneously 13 years after the last CA. N/A A 37 yo F developed dyspnea, fatigue, and palpitations. She had prior history of 2 surgical repairs of an inferior sinus venosus defect (1987, 1991), and CardioSeal umbrella implant with stenting of IVC stenosis (2000). Two RA CA in 2008 for intra-atrial re-entrant tachycardia included cavotricuspid isthmus ablation and a postero-septal line connecting septal scar to the IVC. Ambulatory monitoring in 2020 revealed presumed sinus mechanism with daytime bradycardia of uncertain etiology and rare PVCs. EPS showed RIRA with sinus mechanism connected to posterior and lateral RA, but dissociated from posteroseptal RA extending anteroseptally to include the AVN. The atrial septum demonstrated a slow escape with ventricular conduction and normal HV. Pacing the septum and the appendage captured local tissue but only the former conducted to the ventricle (Figure). A single chamber pacemaker was implanted using a 3830 lead along the interatrial posteroseptum, with resolution of symptoms. To our knowledge, this is the first report of late RIRA arising spontaneously more than a decade after atrial CA. The patient’s symptoms resolved with single chamber atrial pacing.

P718 Unexpected finding during follow up for a suspected arrythmogenic right ventricular cardiomyopathy (ARVC)

Abstract Interatrial communication is the most common congenital defect found in adulthood, being the most common ostium secundum variety (70-80%). Superior and inferior sinus venosus defects are less usual, found in the 5-10% of cases of interatrial communication and frequently associated with anomalous pulmonary venous return (APVR). These defects are located near the junction of the superior (5%) or inferior (&amp;lt;1%) vena cava with the right atrium, which makes them difficult to diagnose by transthoracic ecocardiography (TTE). Case description: A 44-year-old man who is being followed up in our electrophysiology consulting with suspicion of ARVC (suggestive CMR with no gene found) after an episode of ventricular tachycardia (VT) 11 years ago. Asymptomatic since then under treatment with atenolol, except for an episode of chest pain that required a coronary computed tomography which described an image compatible with a patent foramen ovale and normal coronary arteries. During the follow-up a cardio magnetic resonance (CMR) is performed which showed a severely dilated right ventricle with diskinetic areas, no volume changes since last CMR (5 years ago) and preserved ejection fraction. A small interatrial communication located infero-posteriorly in the septum drawed our attention. Estimated QP/QS was 1.4. After this finding, we reviewed the CT made 4 years ago, where a flow from the left atrium to the right atrium could be seen. We decided to ask for both a transthoracic echocardiography (TTE) and a transesophageal echocardiography (TEE). TTE showed normal left ventricle, a dilated right ventricle with preserved function, no valvulopathies and normal pulmonary pressure. Shunt test with agitated saline was slightly positive after Valsalva maneuver, and QP/QS was again 1.4. TTE showed a small interatrial communication measuring 1.9x0.8cm, next to inferior vena cava`s drainage. Since right ventricle dilation could be due both to the atrial septal defect (ASD) and to the dysplasia, the case was discussed in the heart team, and as the defect was small, QP/QS was 1.4 and pulmonary pressure was normal we adopted a conservative approach. Inferior sinus venosus defects are one of the least common atrial septal defects. They are located in the atrial septum immediately above the orifice of the inferior vena cava and are often associated with partial anomalous connection of the right pulmonary veins. This location makes it difficult to see by means of a common TTE or TEE, and usually as in our case multimodal approach can be very helpful. Usually patients with this kind of atrial septal defect (ASD), signs of significant shunt (right ventricular volume overload, QP/QS≥1.5) and systolic PA pressure less than 50% of systemic pressure (with pulmonary vascular resistance less than one third of the systemic vascular resistance) are suggested for surgery. In this case the possibility of two pathologies overlapping makes it challenging for diagnosis and treatment. Abstract P718 Figure. Multimodal imaging for diagnosis.

Feasibility of Device Closure for Multiple Atrial Septal Defects With an Inferior Sinus Venosus Defect: Procedural Planning Using Three-Dimensional Printed Models

Multiple atrial septal defects (ASD) with an inferior sinus venosus defect (SVD) have always been considered to be contraindications for interventional therapy. On the basis of early experience using a patent ductus arteriosus (PDA) occluder for interventional treatment for inferior ASD, this study investigated the feasibility of transcatheter closure of multiple ASDs with an inferior SVD under the guidance of three-dimensional (3D) printed heart models. Between August 2016 and February 2017, five patients who were diagnosed with multiple ASDs with an inferior SVD at the First Affiliated Hospital of Xi'an Jiaotong University underwent cardiac computed tomography (CT) scans and three-dimensional (3D) echocardiography to generate heart disease models by a 3D printing technique. The best occlusion program was determined through a simulated closure on the model. Percutaneous device closure of multiple ASDs with an inferior SVD was performed following the predetermined program, guided only by fluoroscopy. Follow-up included electrocardiography, transthoracic echocardiography, and transoesophageal echocardiography. Three-dimensional (3D) printed models for all five patients were produced successfully. Four (4) patients had a secundum ASD with an inferior sinus venosus ASD, and one patient had a patent foramen ovale (PFO) with an inferior sinus venosus ASD. All patients were successfully treated with interventional therapy. Inferior sinus venosus ASD was percutaneously closed using the PDA occluder, and the additional secundum ASD or PFO in each patient was percutaneously closed using an ASD or PFO occluder at the same time. There was no device embolisation, procedure-related death or pericardial tamponade. During the 1-year follow-up, a minor residual shunt was detected in one patient. The use of 3D printed ASD models provides a useful reference for transcatheter device closure of multiple ASD with an inferior SVD. This approach can provide a new treatment strategy for inferior sinus venosus ASD, which has been considered a contraindication for interventional therapy. However, long-term follow-up in a large number of patients is still warranted.

Anatomy of the atrial septum and interatrial communications.

Deficiencies in the septum separating the two atrial chambers are among the most common of congenital heart malformations. This article reviews the developmental aspects of the partitioning of the primitive atrium into right and left atrial chambers, the anatomical components of the atrial septum, and deficiencies that produce the various types of interatrial communications. Knowledge of the components of the true atrial septum in the developed heart clarifies the morphology of various types of interatrial communications. The oval fossa defect (also termed secundum ASD) is located within the true septum. The patent foramen ovale (PFO) is a tunnel-like passageway between the free edge of the overlapping ovale fossa valve and its muscular rim. Other defects such as superior and inferior sinus venosus defects, coronary sinus defects, and ostium primum defects lie outside the area of the true septum.

A COMMON PRESENTATION OF AN UNCOMMON ATRIAL SEPTAL DEFECT

Inferior sinus venosus defect (SVD) is a rare congenital heart disease involving an interatrial communication outside the confines of the fossa ovalis. It overrides the inferior vena cava and extends into the inferoposterior border of the left atrium, leaving no residual atrial septal tissue at the

The Parasternal Short-Axis View Improves Diagnostic Accuracy for Inferior Sinus Venosus Type of Atrial Septal Defects by Transthoracic Echocardiography

Sinus venosus defects (SVD) of the inferior vena cava (IVC) type, or inferior SVDs, are an uncommon form of atrial communication located outside the confines of the fossa ovalis and involve override of the IVC. Despite numerous studies describing the anatomical and echocardiographic features of the inferior SVD, distinguishing this defect from a large secundum atrial septal defect (ASD) by echocardiography is often challenging. Accurate diagnosis of an inferior SVD and correct differentiation from a secundum ASD is essential for appropriate presurgical planning. Absence of the posterior rim in the parasternal short-axis views may serve as a useful clue in diagnosing inferior SVDs. We sought to determine the utility of using the presence or absence of a posterior atrial rim in the parasternal short-axis view to help distinguish an inferior SVD from a secundum ASD. This sign may help clinch the diagnosis when subcostal imaging is suboptimal. We retrospectively reviewed transthoracic echocardiograms from 15 patients with a known surgical diagnosis of an inferior SVD between 2004 and 2015. The presence or absence of a posterior rim in the parasternal short-axis view was determined by two primary investigators. The posterior rim was also evaluated in 14 patients with a secundum ASD repair as controls. Echocardiograms were then reviewed blindly by attending-level echocardiographers and cardiology fellows in training. Diagnostic accuracy was assessed both with and without the use of the posterior rim criterion. Statistical analysis was used to determine the effect of using the rim criterion on inferior SVD diagnosis. We also reviewed all surgically diagnosed secundum ASDs that were incorrectly diagnosed as inferior SVD by preoperative imaging and determined whether use of the posterior rim criterion would have resulted in the correct diagnosis. The posterior rim was absent in all 15 patients with a surgical diagnosis of inferior SVD and present in all 14 patients with a secundum ASD. For all observers, there was a statistically significant increase in diagnostic accuracy of inferior SVDs with the use of the rim criterion (P<.0001). We noted that secundum ASDs with inferior extension also have persistent posterior rims. The rim criterion correctly classified all large secundum ASDs with inferior extension that were previously misdiagnosed by echocardiogram preoperatively. Absence of the posterior rim ("bald" posterior wall) is a consistent finding in patients with an inferior SVD and distinguishes an inferior SVD from a large secundum ASD with inferior extension. Parasternal short-axis evaluation of the posterior atrial rim is a helpful tool for all levels of physician training in improving diagnostic accuracy for detecting inferior SVDs and in distinguishing them from secundum ASDs.

Inferior sinus venosus and two ostium secundum atrial septal defects is possible to treat with three occluders.

A 73-year-old woman known to have multiple atrial septal defects (mASD) since 2006 rejected cardiac surgery and was referred to our department with signs of right heart failure. Echocardiography demonstrated one defect located in the inferior portion of the atrial septum that led to an overriding inferior vena cava (IVC) – an inferior sinus venosus defect (SVD) and two additional ostium secundum ASDs (ASDII; Figure 1). Furthermore, a significant left-to-right atrial shunt and dilation of the right ventricle were observed.

Surgical repair of inferior sinus venosus defects: a novel approach with unsnared inferior vena cava.

BackgroundInferior sinus venosus defects (SVD) are very rare and difficult to image from transthoracic echocardiography. Surgical errors were occasionally reported in the repair of inferior SVDs.ResultsThe authors have operated on 12 inferior SVD patients using bicaval cannulation with unsnared inferior vena cava (IVC) and proved successful.ConclusionThis technique guaranteed a better exposure of surgical field and facilitate identifying the anatomical relationship between lower part of the SVD and IVC orifice, thus avoiding postoperative IVC - left atrial shunt and other surgical mistakes.

Cine acquisition strategies for visualizing atrial septal defects by CMR

Background Atrial septal defects (ASDs) may escape detection before adulthood, particularly those in unusual locations, which may elude echocardiographic visualisation. CMR is established for the quantification of shunt flow and, if appropriately acquired, can provide clear visualisation of ASDs and adjacent structures to inform decisions regarding intervention. Our objective is to recommend cardiovascular magnetic resonance (CMR) cine acquisition strategies suitable for the visualisation of the less common as well as the common types of ASD. Methods In the CMR Unit of a tertiary referral centre for adults with congenital heart disease we retrospectively reviewed the CMRs of patients with unoperated ASDs over a 3 year period to assess the suitability of cine acquisition strategies for the visualisation of different types of ASD and any associated anomalies of pulmonary venous connection. Results 157 patients with unoperated ASDs had CMR studies in 3 years. If already suspected, we had routinely acquired an ‘atrial stack’ of cines, meaning a contiguous stack, 5mm thick, parallel to the routine ventricular short axis (SA) stack, stepping backward from the basal ventricular plane until the superior vena cava was identifiable. This orientation visualized ostium secundum ASDs well (n=117), and was also good for inferior sinus venosus defects (n=2) and unroofed coronary sinus (n=3). In superior sinus venosus defects (n=21), a transaxial cine stack was found to give clearer visualisation of both the defect and any associated anomalous pulmonary vein connection(s) (n=20, plus 4 anomalous connections identified with secundum ASDs). The transaxial cine orientation was also the more suitable one for atrio-ventricular septal defects (AVSD, n=15) as it depicted insertions of the A-V valve leaflets adjacent to the defect(s). Conclusions In patients with suspected ASDs, we recommend the acquisition of an atrial SA cine stack and a transaxial cine stack that covers atrial to aortic arch levels. Additional oblique cines aligned with the defect can supplement these, as indicated in the table. Funding None.

Inferior Sinus Venosus Defects: Anatomic Features and Echocardiographic Correlates

Inferior sinus venosus defects (SVDs) are rare imperfections located in the inferior portion of the atrial septum, leading to an overriding inferior vena cava (IVC) and an interatrial connection. These defects have increased risk of anomalous pulmonary venous return (PAPVR) and often are confused with secundum atrial septal defects (ASDs) with inferior extension. The authors sought to review their experience with inferior SVDs and to establish at their institution an echocardiographic definition that differentiates inferior SVDs from secundum ASDs with inferior extension. The study identified 161 patients 1.5 to 32 years of age who had undergone repair of a secundum ASD with inferior extension or inferior SVD over the preceding 10 years. All surgical notes, preoperative transthoracic echocardiograms (TTEs), and preoperative transesophageal echocardiograms (TEEs) were reviewed. Based on the surgical notes, 147 patients were classified as having a secundum ASD (147/161, 91 %) and 14 patients (9 %) as having an inferior SVD. The study identified PAPVR in 7 % (1/14) of the patients with inferior SVDs and 3.5 % (5/14) of the patients with secundum ASDs. Surgical diagnosis and preoperative TTE correlated for 143 (89 %) of the 161 patients. Using a strict anatomic and echocardiographic definition with a blinded observer, the majority of the defects (14/18, 78 %) were reclassified correctly after review of their TTE images, and 100 % of the defects were correctly reclassified after TEE image review. Accurate diagnosis of inferior SVDs remains challenging. The data from this study demonstrate that use of a strict anatomic and echocardiographic definition (a defect that originates in the mouth of the IVC and continues into the inferoposterior border of the left atrium, leaving no residual atrial septal tissue at the inferior margin) allows for accurate differentiation between secundum ASDs with inferior extension and inferior SVDs. This differentiation is extremely important in planning for surgical versus device closure of these rare defects.

Surgical repair after ineffective device closure of an inferior sinus venosus defect

A young woman presented with severe heart failure symptoms 4 years after percutaneous device closure of an atrial septal defect (ASD). There was residual left-to-right shunting, and the device was obstructing the inferior caval vein and tricuspid valve flow. Intraoperatively, the ASD was shown to be an inferior sinus venosus defect, and reconstruction of the mitral valve and the posterior wall of the left atrium was required. This case emphasizes the importance of appropriate patient selection for interventional ASD closure and demonstrates that patients with less-than-optimal results should be referred for surgical correction early, before the device produces secondary damage to atrial structures and atrioventricular valves.

Outcomes of inferior sinus venosus defect repair

Inferior sinus venosus defect is an unusual form of interatrial communication with few published data on surgical outcomes. We sought to compare outcomes of surgical repair of inferior sinus venosus defect with those of large secundum atrial septal defects. Patients undergoing surgical closure of an isolated interatrial defect were reviewed, and those with inferior sinus venosus defect were identified on the basis of predetermined anatomic criteria. For each case, 2 controls with secundum atrial septal defect, matched for age and year of surgery, were selected. Technical outcome scores and other perioperative outcomes were compared. Compared with the secundum atrial septal defect group (n = 90), the inferior sinus venosus defect group (n = 45) had worse technical outcome scores (P = .02), a higher rate of reintervention (9% vs 1%, P = .04), longer median total cardiopulmonary bypass (48 vs 39 minutes, P < .001) and crossclamp (29 vs 20 minutes, P < .001) times, and were more likely to stay more than 1 day in the intensive care unit (20% vs 8%, P = .04) and more than 3 days in the hospital (29% vs 13%, P = .03). Only 16 (36%) of the patients with inferior sinus venosus defect had a correct diagnosis preoperatively. Patients with an incorrect diagnosis had worse technical outcome scores than the secundum atrial septal defect group (P = .003), whereas those with a correct diagnosis had scores similar to those of the secundum atrial septal defect group (P = .55). Compared with patients with secundum atrial septal defect, patients with inferior sinus venosus defect have more residual defects and longer durations of cardiopulmonary bypass and hospitalization. Rates of misdiagnosis of inferior sinus venosus defect are high and associated with worse technical outcome scores. Accurate preoperative diagnosis of this lesion may lead to improved outcomes.

Magnetic Resonance Imaging-Aided Diagnosis of an Inferior Sinus Venosus Defect: A Case Report

Magnetic Resonance Imaging-Aided Diagnosis of an Inferior Sinus Venosus Defect: A Case Report

Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

We sought to define the inferior sinus venosus defect anatomically and document successful surgical approaches. We identified all patients previously given a diagnosis of an inferior sinus venosus defect at the Hospital for Sick Children, Toronto, Canada, between 1982 and 2005 by interrogating the cardiology and cardiac surgery databases. We included those having interatrial communications in which 1 or more of the right pulmonary veins drained to the inferior caval vein but retained connection with the left atrium, the rims of the oval fossa, and the walls of the coronary sinus, both being intact. We identified 11 children who had an interatrial communication meeting the criteria for and undergoing surgical repair of an inferior sinus venosus defect. Median age was 1.2 years; 6 (55%) subjects were male, and none were cyanotic. Transthoracic echocardiographic analysis was performed preoperatively in all children, revealing right ventricular dilation in all. Surgical repair was accomplished with a pericardial patch. A complex baffle was needed in 3 children to maintain unobstructed inferior caval and pulmonary venous return. The echocardiographic diagnosis was complete in only 5 patients, but all diagnoses were correct since the year 2000. In all children the observations at surgical intervention showed that the defect was a venoatrial communication involving drainage of the right pulmonary veins to the inferior caval vein while retaining connection to the left atrium. Transthoracic echocardiographic analysis should remain the modality of choice for diagnosis of the inferior sinus venosus defect. We report excellent surgical results with a patch or baffle, correctly redirecting the anomalous venoatrial connections.