Superior Sinus Venosus Atrial Septal Defect Research Articles

Kiosk 2Q-TA-05 - Transcatheter Treatment of Superior Sinus Venosus Atrial Septal Defect (SVASD) with Partial Anomalous Pulmonary Venous Drainage (PAPVD): The Critical Role of 3D Modelling

Kiosk 2Q-TA-05 - Transcatheter Treatment of Superior Sinus Venosus Atrial Septal Defect (SVASD) with Partial Anomalous Pulmonary Venous Drainage (PAPVD): The Critical Role of 3D Modelling

Biatrial drainage of right superior vena cava with left superior vena cava: A diagnostic conundrum

Biatrial drainage of the right superior vena cava (R-SVC) is a rare entity, with fewer than 40 reported cases.1,2 There are several anatomical considerations, but only one case with associated persistent left superior vena cava (PLSVC) has previously been reported.1 When the R-SVC drains into both atria, either due to an abnormal connection, malposition of the atrial septum, or a superior sinus venosus atrial septal defect (SV-ASD), it may result in a right-to-left shunt with potentially significant consequences.

TCT-349 Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defect (SVASD) Using Self-Expandable Aortic Stent Graft: A Case Series

TCT-349 Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defect (SVASD) Using Self-Expandable Aortic Stent Graft: A Case Series

Superior sinus-venosus atrial septal defect complicated by partial anomalous pulmonary venous connection detected by repeated transthoracic echocardiography in an adult.

Superior sinus-venosus atrial septal defect complicated by partial anomalous pulmonary venous connection detected by repeated transthoracic echocardiography in an adult.

Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defects as an Alternative to Surgical Treatment

BackgroundThe superior sinus venosus atrial septal defect (SVASD) is characterized by deficiency of the common wall between the superior vena cava (SVC) and the right upper pulmonary vein (RUPV), which is no longer committed to the left atrium. ObjectivesThis study sought to evaluate the potential for redirecting the SVC and RUPV flow to the right and left atria, respectively, by implantation of a covered stent in the SVC. MethodsReview of 48 consecutive adult SVASD patients undergoing assessment for correction. Pre-procedural evaluation included cross-sectional imaging and ex vivo simulation using printed or virtual 3-dimensional models. ResultsTranscatheter correction was performed in 25 patients, with a further 6 awaiting stent implantation. Only 8 patients were deemed technically unsuitable. The procedure involved balloon test inflation in the anticipated stent landing zone with simultaneous transesophageal echocardiography and pulmonary venography to confirm defect closure and unobstructed pulmonary venous drainage, followed by deployment of a 10-zig covered Cheatham platinum stent. Stents of lengths between 5 and 8 cm were implanted. A second, uncovered stent was used for anchoring in 9 patients. The RUPV was protected with a high-pressure balloon during stent implantation to prevent pulmonary venous obstruction in 4 patients. The median follow-up period was 1.4 (interquartile range: 0.8 to 1.7) years, with no mortality. Stent embolization occurred in 1 patient; another required drainage of hemopericardium. Cardiac computed tomography after 3 months confirmed unobstructed pulmonary venous return. At latest follow-up, a residual shunt was present in 1 patient. ConclusionsTranscatheter correction of SVASD may be considered as an alternative to surgery in a substantial proportion of patients.

P649 Before and after: sinus venosus atrial septal defect

Abstract Introduction The diagnosis of sinus venosus atrial septal defect (SVASD) is complex and requires special imaging. Surgery is the conventional treatment; however, transcatheter repair may become an attractive option. Case report A 60 year-old woman was admitted to the cardiology department with several episodes of paroxysmal atrial flutter, atrial fibrillation and atrioventricular nodal reentrant tachycardia. She reported a 10-year history of occasional palpitations which had not been studied. A transthoracic echocardiography revealed severe right ventricle dilatation and moderate dysfunction. Right volume overload appeared to be secondary to a superior SVASD with partial anomalous pulmonary venous drainage. A transesophageal echocardiography confirmed the diagnosis revealing a large SVASD of 16x12 mm (Figure A) with left-right shunt (Qp/Qs 2,2) and two right pulmonary veins draining into the right superior vena cava. Additionally, it demonstrated coronary sinus dilatation secondary to persistent left superior vena cava. CMR and cardiac CT showed right superior and middle pulmonary veins draining into the right superior vena cava 18 mm above the septal defect (Figures B and C). After discussion in clinical session, a percutaneous approach was planned to correct the septal defect and anomalous pulmonary drainage. For this purpose, anatomical data obtained from CMR and CT was needed to plan the procedure. During the intervention two stents graft were deployed in the right superior vena cava. The distal stent was flared at the septal defect level so as to occlude it while redirecting the anomalous pulmonary venous flow to the left atrium (Figure D). Control CT confirmed the complete occlusion of the SVASD without residual communication from pulmonary veins to the right superior vena cava or the right atrium (Figure E). Anomalous right superior and middle pulmonary veins drained into the left atrium below the stents. Transthoracic echocardiographies showed progressive reduction of right atrium and ventricle dilatation. The patient also underwent successful ablation of atrial flutter and intranodal tachycardia. She is currently asymptomatic, without dyspnea or arrhythmic recurrences. Conclusions In this case, multimodality imaging played a key role in every stage of the clinical process. First, it provided the diagnosis and enabled an accurate understanding of the patient’s anatomy, particularly of the anomalous pulmonary venous connections. Secondly, it allowed a transcatheter approach by supplying essential information to guide the procedure. Finally, it assessed the effectiveness of the intervention and the improvement in cardiac hemodynamics during follow-up. Abstract P649 Figure.

Rapid Prototyping and Image Fusion Guidance for Transcatheter Closure of Superior Sinus Venosus Atrial Septal Defect

Sinus venosus atrial septal defect (SVASD) accounts for 5–10% of all ASDs. Partial anomalous pulmonary venous connection (PAPVC) and pulmonary hypertension (PH) in patients with SVASD increases the risk for surgical or transcatheter intervention. Closure is preferred over medical therapy. Transcatheter closure is challenging due to complex anatomy, difficulty in evaluating suitability for intervention, and lack of dedicated devices. We present a systematic approach using multimodality imaging and 3D printing for transcatheter closure of superior SVASD with PAPVC using a custom-made CP Stent®. A 53-year-old with history of drug abuse presented with NYHA Class III symptoms, hypoxemia, atrial flutter, and PH (MPAP of 30 mmHg) requiring multidrug therapy. Echocardiogram raised suspicion of superior SVASD, confirmed by cardiac CT and CMR. Multidisciplinary team ruled out surgery due to high risk and recommended transcatheter closure, if feasible. CT and CMR demonstrated a 20 × 18 mm superior SVASD with PAPVC: right upper (RUPV) and middle pulmonary veins draining into the superior vena cava (SVC). To understand spatial relationships, a 3D model was printed with 1.5-mm transparent and flexible walls to deploy a non-sterile prototype of the stent in the SVC. Rotational angiogram was performed to test feasibility of in-vivo deployment in view of stent foreshortening (from 110 to 70 mm) and differential widening of the SVC: 23 mm at the junction of the innominate vein; 35 mm at the atrial end. Transcatheter closure of superior SVASD with PAPVC in patients with PH is feasible in suitable cases using a systematic approach with multi-modality imaging and 3D printing.

Early experience of transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage

Superior sinus venosus atrial septal defect (SVASD) is commonly associated with partial anomalous pulmonary venous drainage (PAPVD). We aim to describe the first series of percutaneous SVASD and PAPVD correction using a two-step simulation for procedural planning. Patients with SVASD and right PAPVD with a clinical indication for correction were selected. They underwent an ex vivo procedural simulation on a 3D-printed model followed by an in vivo simulation using balloon inflation in the targeted stent landing zone. The percutaneous procedure consisted in deploying a 10 Zig custom-made covered stent in the SVC-RA junction. Nine patients were referred for pre-procedural evaluation and were deemed suitable for percutaneous correction. The procedure was successful in all patients with no residual interatrial shunt and successful redirection of the pulmonary venous drainage to the left atrium. At a median clinical follow-up of 8.1 months (2.6–19.8), no adverse events were noted and all patients showed clinical improvement. During follow-up, transthoracic echocardiography and MDCT in 4 patients or invasive angiography in one patient demonstrated a patent SVC stent, no residual SVASD and unobstructed PV drainage in all patients. In selected patients using a two-staged simulation strategy, percutaneous correction of SVASD with PAPVD is feasible, safe and led to favorable midterm outcomes.

Early experience of transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage.

Superior sinus venosus atrial septal defect (SVASD) is commonly associated with partial anomalous pulmonary venous drainage (PAPVD). We aimed to describe the first series of percutaneous SVASD and PAPVD correction using a two-step simulation for procedural planning. Patients with SVASD and right PAPVD with a clinical indication for correction were selected. They underwent an ex vivo procedural simulation on a 3D-printed model followed by an in vivo simulation using balloon inflation in the targeted stent landing zone. The percutaneous procedure consisted in deploying a 10-zig custom-made covered stent in the SVC-RA junction. Five patients were referred for preprocedural evaluation and were deemed suitable for percutaneous correction. The procedure was successful in all patients with no residual interatrial shunt and successful redirection of the pulmonary venous drainage to the left atrium. At a median clinical follow-up of 8.1 months (2.6-19.8), no adverse events were noted, and all patients showed clinical improvement. During follow-up, transthoracic echocardiography and multidetector cardiac tomography in four patients or invasive angiography in one patient demonstrated a patent SVC stent, and no residual SVASD and unobstructed PV drainage in all patients. In selected patients using a two-stage simulation strategy, percutaneous correction of SVASD with PAPVD is feasible and safe, and led to favourable short-term outcomes.

Iatrogenic Narrowing of Inferior Vena Cava after Atrial Septal Defect Closure: The Role of Intraoperative Echocardiography

ABSTRACT Single-patch closure of a superior sinus venosus atrial septal defect (SVASD) may be complicated with narrowing of inferior vena cava (IVC). If the pressure gradient across the narrowed portion of superior vena cava (SVC) exceeds 6 mm Hg, widening of SVC lumen is considered. No such guidelines are available in literature for narrowing of inferior vena cava in cardiac surgical patients. We describe an incident of inferior narrowing of inferior vena cava in a 10-year-old girl, who was operated for closure of an inferior type of ostium secundum ASD (OSASD). There was hepatic venous dilatation accompanied with gradient of 3 mm Hg at the junction of inferior vena cava and right atrium (RA). The cardiopulmonary bypass was reestablished and the surgical closure of the septal defect was revised to rectify the problem. How to cite this article Gadhinglajkar S, Sreedhar R, Lovhale P, Mathew T, Tapdia N. Iatrogenic Narrowing of Inferior Vena Cava after Atrial Septal Defect Closure: The Role of Intraoperative Echocardiography. J Perioper Echocardiogr 2015;3(2):48-51.

Superior Sinus Venosus Atrial Septal Defect: Overview of Surgical Options

Background: Superior sinus venosus atrial septal defect (SV-ASD) is an interatrial defect with partial anomalous pulmonary venous connection (PAPVC) draining into the right atrium. The principle for SV-ASD surgical repair involves redirecting the pulmonary venous blood back to the left atrium and closure of the atrial septal defect without compromising the adjoining structures. Methods: We reviewed English literature relating to this topic via a MEDLINE and Google scholar search using the following terms: surgery for sinus venosus atrial septal defect (SV-ASD), surgery for partial anomalous pulmonary venous connection (PAPVC) and complications of the surgery. Results: A total of 910 consecutive cohort patients with different surgical options between 1984 to 2012 were analyzed. The breakdown showed that 291(32.0%) had single-patch, 275(30.2%) had Warden’s procedure, 185(20.3%) had a double-patch and 159(17.5%) had use of autologous right atrial appendage for the anastomosis. The total follow-up was between 0.008-30 years. Sinus node dysfunction (SND) was the commonest complication occurring mostly in patients who had double-patch technique 16(8.6%) followed by 15(5.5%) patients with single-patch technique, 7(4.4%) patients in whom right atrial appendage was used and 5(1.8%) patients with Warden’s procedure. Venous obstruction occurred in 22(7.7%) patients with SP, 13(5.1%) patients with Warden’s Procedure and 5(2.7%) patients with double-patch. Conclusion: The two major complications, SND and venous obstruction, assumed a see-saw approach as surgical maneuvers avoiding venous obstruction encouraged SND and vice versa. However, adopting surgical options which avoided incision across the Cavoatrial junction attenuated all the complications.

MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects

Purpose of this survey was to estimate the value of MRI for the assessment of the anatomical and functional features of sinus venosus atrial septal defect (SVD). This prospective study included 13 surgically proven cases of SVD out of 81 subjects submitted to MRI due to inconclusive transthoracic echocardiography (TTE) or suspicion of high intracardiac and/or extracardiac shunt volumes based on echocardiographic findings. MRI examination included cine SSFP sequences, contrast-enhanced 3D gradient-echo (GE) sequences for MR angiography (MRA) and phase-contrast flow-measurements. MRI revealed nine patients with a superior and four with an inferior SVD. Anomalous pulmonary venous drainage (APVD) was observed only in subjects with a superior SVD, and it was right-sided in all cases. All MRI and MRA results for the SVD patients were confirmed intraoperatively. The Correlation coefficient between MR flow measurements and cardiac catheterisation was 0.94 (P<0.0001). According to MRI the rest of the subjects (n=68) presented a secundum ASD, whereas in 18% an APVD coexisted. The latter MR outcomes concurred with the cardiac catheterisation (n=56) and operative (n=12) results. MRI provides a reliable, non-invasive method for evaluation of SVDs, APVDs and shunt quantification.

Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect

Superior type of sinus venosus atrial septal defect (SVASD) is invariably associated with the unroofing of right upper pulmonary vein (RUPV). Warden procedure and pericardial patch repair with rerouting of the RUPV are commonly performed operations for the superior SVASD. Both operations involve the risk of obstruction to the flow of superior vena cava or rerouted pulmonary vein in the postoperative period. The sinus venosus defects are well visualized on the transesophageal echocardiography (TEE) because of the proximity of the TEE probe to these structures. We are reporting two cases operated for the superior SVASD with unroofed RUPV, highlighting the intraoperative echocardiographic features before and after the surgery.