Sinus Venosus Atrial Septal Defect Research Articles

Delayed Diagnosis of Right Heart Failure Due to Sinus Venosus Atrial Septal Defect, Persistent Left Superior Vena Cava and Partial Anomalous Pulmonary Venous Return

Sinus venosus atrial septal defects (SVASD) are uncommon and often underdiagnosed as they are visualised poorly on transthoracic echocardiography (TTE). A 59-year-old man was referred following worsening dyspnoea. A TTE demonstrated dilated right chambers, with torrential tricuspid regurgitation and a dilated coronary sinus. Agitated saline test raised the suspicion of both persistent left superior vena cava (PLSVC) and an intracardiac shunt, despite interrogation of the interatrial septum not demonstrating any defects. CT imaging of the thorax was performed revealing a superior SVASD alongside a PLSVC, and partial anomalous pulmonary venous return. SVASD are considerably uncommon, compared to other types of atrial septal defects (ASD). TTE has poor sensitivity in diagnosing SVASD, highlighting the importance of multimodality imaging in such cases. The purpose of this case report is to highlight unique features demonstrated on TTE, especially during the agitated saline study, which showcased the presence of a PLSVC alongside an intracardiac shunt that was undetected through conventional TTE alone.

Percutaneous closure of a superior sinus venosus atrial septal defect with partial pulmonary anomalous venous drainage: An option also for children.

Percutaneous closure of a superior sinus venosus atrial septal defect with partial pulmonary anomalous venous drainage: An option also for children.

Sinus venosus atrial septal defect presenting with platypnea-orthodeoxia syndrome in the setting of COVID-19 infection.

Platypnea-orthodeoxia syndrome is characterised by hypoxemia and dyspnoea while upright that resolves when supine, typically associated with an interatrial communication. We present a case of platypnea-orthodeoxia syndrome associated with a sinus venosus atrial septal defect in a patient with multiple possible aetiologies of hypoxemia, including COVID-19 infection. Cardiac catheterisation with provocative maneuvers confirmed the diagnosis and symptoms resolved following defect closure. We suggest that in patients with platypnea-orthodeoxia syndrome, it is useful to obtain haemodynamic data while supine and upright.

Atrial septal defect and zero positive end-expiratory pressure: A case report

This case report aims to investigate the impact of zero Positive End-Expiratory Pressure (PEEP) on the respiratory parameters of a patient following atrial septal defect repair who requires mechanical ventilation. We present the case of a 33-year-old patient who had been experiencing recurrent chest infections accompanied by copious cough with greenish sputum since 2009, without experiencing shortness of breath. The patient had no significant childhood illnesses and had initially been treated with frequent antibiotics at a local health center, which did not lead to symptom improvement. Subsequently, the patient was referred to a tertiary level University Hospital and underwent a series of diagnostic investigations. Transesophageal echocardiography revealed a defect in the superior and posterior location of the interatrial septum, measuring approximately 16mm with left-to-right flow. This defect was situated at the insertion of the superior vena cava, consistent with a superior sinus venosus atrial septal defect. The examination also revealed a moderately dilated right atrium and mild-to-moderate tricuspid regurgitation. Consequently, the patient underwent intracardiac repair of the atrial septal defect on cardiopulmonary bypass. In light of the ongoing increase in symptoms, right ventricle remodeling, and pulmonary artery pressure as the patient ages, it is essential to consider recommencing atrial septal defect closure early after diagnosis, regardless of the presence of symptoms..

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

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience.

Sinus venosus atrial septal defects present a wide variety of anatomical features and are frequently associated with partial anomalous pulmonary venous drainage of one or more right pulmonary veins. Surgical correction used to be the standard treatment. In recent times, transcatheter correction of superior sinus venosus atrial septal defects has come into vogue. The transcatheter closure of these defects with covered stents at a tertiary care centre in Oman between 2018 and 2023 is reported.

Oclusão percutânea da comunicação interatrial do tipo seio venoso com stent coberto: a importância dos métodos de imagem para impressão de modelos tridimensionais e a melhor tomada de decisão clínica

A superior sinus venosus atrial septal defect is usually accompanied by an anomalous venous connection to a right superior or middle pulmonary vein or veins, draining directly into the superior vena cava or even into the cavoatrial junction. This is a case report of a 62-year-old female patient, diagnosed with a sinus venosus atrial septal defect, with overload of the right chambers, for whom a percutaneous occlusion procedure was planned, using a previous cardiovascular tomography and, sequentially, a three-dimensional anatomical study, with the publicly available software 3D Slicer. In addition, a resin model was printed for inspection and simulation of a stent implantation. The patient was treated percutaneously with a 60-mm covered Chetham-Platinum stent, with total occlusion of the defect, absence of residual shunts, and draining flow from the right superior pulmonary vein to the left atrium, through a posterior communication between the atria, a sine qua non prerequisite to perform this type of procedure. Planning of the percutaneous occlusion procedure of the sinus venosus atrial septal defect involves careful evaluation of imaging tests. The printing of virtual or physical models, derived from computed tomography angiography of the heart, is essential for a detailed study of the defect and associated anatomical structures, minimizing the occurrence of complications.

Abstract 13138: Outcomes of Surgical Repair for Sinus Venosus Atrial Septal Defect

Introduction: Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5-10% of all atrial septal defects. Although surgical closure is the standard treatment for SVASD, data on outcomes have been confined to small cohorts. Thus, we conducted a systematic review and meta-analysis of outcomes of SVASD repair. Methods: A comprehensive search of PROSPERO, MEDLINE, EMBASE, and Cochrane Library databases was conducted. The primary outcome was mortality. Secondary outcomes encompassed atrial fibrillation, sinus node dysfunction, pacemaker insertion, cerebrovascular accident (CVA), reoperation, residual septal defect, superior vena cava (SVC) obstruction, and reimplanted pulmonary vein obstruction. Pooled incidences of outcomes were calculated using a random effects model. Results: Forty studies involving 1,292 patients who underwent SVASD repair were included. The majority were male (59.4%), with 92.9% presenting with associated pulmonary anomalous venous connection. The weighted mean ages were 18.6 ± 12.5 years, and the overall weighted mean follow-up period was 8.6 ± 10.4 years. In-hospital mortality rate was 0.24%, with a 30-day mortality of 0.56% reported in 720 patients. Incidences of atrial fibrillation, sinus node dysfunction, pacemaker insertion, and CVA over the long-term follow-up were 2% (1-3%), 4% (2-6%), 2% (1-2%), respectively. Reoperation, residual septal defect, SVC obstruction, and reimplanted pulmonary vein obstruction occurred in 1% (0-2%), 2% (2-2%), 1% (1-1%), and 2% (2-2%) surgeries, respectively. Conclusions: This is the first comprehensive analysis of outcomes following surgical repair of SVASD. The findings affirm the safety and effectiveness of surgical intervention, establishing a reference point for evaluating emerging transcatheter therapies. Comparable safety and efficacy profiles to surgical repair are essential for widespread adoption of transcatheter treatments.

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Sinus venosus atrial septal defect in adults: Surgical repair using partial thoracoscopic approach

Sinus venosus atrial septal defect in adults: Surgical repair using partial thoracoscopic approach

Sinus node dysfunction during transcatheter assessment and stent correction of sinus venosus atrial septal defects.

Covered stent correction of a superior sinus venosus atrial septal defect is increasingly performed as an alternative to surgical repair.While sinus node dysfunction requiring pacemaker implantation may be required after surgical repair, this has not been previously reported after covered stent implantation. We reviewed the experience in two interventional centers.Balloon inflation in the superior vena cava was used to confirm the anomalous pulmonary vein drainage would be unobstructed after stent implantation. During balloon testing in 62 consecutive patients, we assessed gradients across the pulmonary vein to left atrium while monitoring the rhythm. We observed the outcomes after covered stent correction in 51 patients.In a single patient, significant bradycardia and pauses developed on repeat balloon testing and the procedure was abandoned without stent implantation. In another patient, there was no sign of sinus node dysfunction during balloon testing but several hours after stent implantation, the patient became symptomatic from sinus bradycardia and pauses and had a pacemaker implanted 3 days later. Over a year later there are some signs of improvement in sinus node function.While sinus node dysfunction has not been described previously during balloon testing or after stent implantation, this report demonstrates for the first time that it may occur. Larger registries are therefore required to monitor for this uncommon complication.

Case Report: Removal of septal occluder device and closure of atrial septal defect with pulmonary vein flow redirection

BACKGROUND: Atrial septal defect represents 10 to 15% of congenital heart disease and is more frequent in women with a 2:1 ratio. Sinus venosus atrial septal defect is usually associated with partial anomalous pulmonary venous drainage from the right superior pulmonary vein to the right atrium directly or indirectly through the superior vena cava. Rarer still, is the inferior sinus venosus, which may be associated with partial anomalous venous drainage from the right inferior pulmonary vein. CASE REPORT: 13-year-old patient with a history of intervention for interatrial defect closure with Amplatzer occluder at the age of six. He was brought to receive medical attention for fatigue and dyspnea that had been progressing for 2 years. Cardiac CT reported partial anomalous connection of pulmonary veins and echocardiogram reported a residual atrial septal defect of 10 mm and mild to moderate pulmonary hypertension. Based on the findings, surgical treatment was decided. EVOLUTION: In correction surgery, we observed Amplatzer occluder incorrectly positioned, the device was removed and flow was redirected from right pulmonary veins to left atrium with bovine pericardium patch. After the surgical intervention, the patient had a favorable evolution without no post-surgical complications. At follow-up, 15 days after medical discharge, the patient was asymptomatic, and a cardiac CT scan showed the corrected interatrial defect. CONCLUSION: The treatment of atrial septal defect should be individualized, since it will depend on the size of the defect, the age at the time of diagnosis, the magnitude of the shunt, the severity of the symptoms and the presence of other conditions. If the defect requires repair, it is important to weigh the benefits of choosing percutaneous closure vs. surgical intervention to avoid the need for reintervention, as in the present case. Closure and redirection of pulmonary vein flow with bovine patch is a safe and effective option.

P113 TRANSCATHETER CORRECTION OF RESIDUAL INFERIOR SINUS VENOSUS DEFECT AFTER SURGICAL CLOSURE

Abstract A 21–year old male who underwent surgical closure of an isolated inferior type sinus venosus atrial septal defect (SV–ASD) at 6 years of age was admitted to our institute for acute superior limb hypotonia and dysarthria, with brain computed tomography scan positive for an ischaemic event. The contrast transcranial Doppler (CTD) was positive for right–to–left shunt (RLS). The transesophageal echocardiogram (TOE) revealed a small ASD (3.8 mm) near the inferior vena cava (IVC) with a small baseline left–to–right shunt and severe RLS after Valsalva’s Manovre (>20 bubbles) (fig.A–B). No clear intra–cardiac source of embolization was detected on workup. After a multidisciplinary discussion, percutaneous closure of residual ASD was planned. The main challenge was represented by inadequate inferior–posterior rim towards the IVC (<5 mm). A Figulla Flex II ASD Occluder with the smallest waist of 4 mm was implanted with no evidence of residual intraprocedural shunt; no intraprocedural complications were observed (fig.C). The 1 month follow–up CTD was negative for RLS. Conclusions Residual ASD is a rare late complication after surgical closure of the ASD(1). We describe the case of a successful transcatheter closure of a small ASD in the presence of a deficient posterior–inferior rim which, in general, is considered an unfeasible anatomy for percutaneous procedure (2). The appropriate choice of treatment for a residual ASD should be carefully considered on a case–by–case basis. Reference 1) S. Ogura;Y.Takaya1; T. Akagi; K. Nakagawa; H. Ito.Percutaneous closure of residual atrial septal defect after surgical closure. Cardiovascular Intervention and Therapeutics (2021) 36:256–259 2) M. Chessa; G. Butera; A. Giamberti; R. M. Bini; M.Carminati. Transcatheter Closure of Residual Atrial Septal Defects After Surgical Closure. Journal of Interventional Cardiology vol.15, No. 3,2002

C47 ADAPTATION OF THE PULMONARY CIRCULATION AND OF THE RIGHT HEART IN INTRACARDIAC INVETERATE SHUNTS: UNDER PRESSURE OR LET IT FLOW?

Abstract Congenital heart disease can be associated with pulmonary hypertension (PH) development and heart right remodelling. Nevertheless, the adaptation of the pulmonary circulation and of the right heart can be extremely heterogeneous, so that an accurate diagnostic work up may be advisable when an intracardiac shunt is suspected. Case Presentation A 53 years old woman, with no relevant clinical history and asymptomatic, was incidentally found to have clinical, ECG and echocardiographic signs of PH, and was referred to our Center. Diagnostic and Therapeutic Work Up Echocardiography showed: normal left ventricle (LV 3D volumes 88/35 ml), mild left atrial dilatation (39 ml/m2); severe right chambers dilatation (RV 3D 347/124 ml, EF 3D 62%, AD 116 ml/m2), moderate tricuspid regurgitation with a regurgitant jet velocity of 2.9 m/s. Right heart catheterization showed normal pulmonary pressures at rest (mPAP 20, PAWP 5 mmHg), extremely high pulmonary blood flow (Qp 29 L/min, calculated with the direct Fick method), with normal pulmonary vascular resistance (PVR 0.5 WU). During the oxymetric run, a step up in hemoglobin saturation between superior vena cava and right atrium (from 74 to 97%) was found, with a Qp/Qs ratio of 5.5. During exercise, Qp reserve was normal, suggesting a normal contractile reserve function of RV despite the extreme dilatation. Additionally, the patient did not develop exercise PH and exercise capacity was normal, based on a measured oxygen consumption (VO2) at peak of 24 ml/kg/min (104% of predicted). Angio–CT of the chest was performed, confirming the presence of a sinus venosus atrial septal defect. The patient was then referred for surgical correction. Conclusion Echocardiography signs are useful to direct correctly diagnostic work up. A severe RV dilatation with normal systolic function in an asymptomatic middle age woman should raise the possibility of an intracardiac shunt. Despite a large intracardiac shunt with a high Qp/Qs ratio for decades, PVR was normal, confirming that individual predisposition may play a role in the development of PH in pre–tricuspid shunts. Exercise test showed an optimal RV reserve, suggesting a low cardiac surgery risk.

Totally Endoscopic Surgery for Repairing Sinus Venosus Atrial Septal Defect Using Double-Patch Technique on Beating Heart.

Minimally invasive cardiac surgery has not been widely applied in repairing sinus venosus atrial septal defect (SV-ASD). Most were minithoracotomy using the single-patch technique for patients with anomalous pulmonary veins (APVs) connecting to the superior vena cava-right atrium (SVC-RA) junction. It is unclear whether patients with APVs draining high to the SVC can be repaired safely and effectively through port access. From May 2019 to October 2022, 11 consecutive SV-ASD patients with APVs connecting high to the SVC were enrolled in this prospective study. One 12 mm port and 2 trocars (5.5 mm and 10 mm) were established. The pleural and pericardial spaces were filled with CO2. The SVC was snared just below the azygos vein. The RA incision was longitudinally extended along the SVC-RA junction to the SVC. The bovine pericardial patches were used to redirect the APV flow to the left atrium through the ASD and to enlarge the SVC and the SVC-RA junction. There were no early or late deaths and no reoperations. The concomitant procedures included 5 patients (45.5%) with patent foramen ovale closure, 2 with ASD extension, and 3 with tricuspid valve repair. No endoscopic failure was recorded. The average cardiopulmonary bypass and operative times were 96 (23) min and 190 (30) min, respectively. No cases of venous stenosis or sinus node dysfunction were noted during follow-up of 16.4 ± 12.2 months. SV-ASD with the APVs draining high to the SVC could be repaired safely and effectively through port access with a double-patch technique.

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. Between March 2010 and December 2020, 58patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2cases, the procedure was unsuccessful; the remaining 34were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

Partial anomalous pulmonary venous return with sinus venosus atrial septal defect: a case report

Partial anomalous pulmonary venous return with sinus venosus atrial septal defect: a case report

364 - Partial anomalous pulmonary venous return in the setting of an inferior sinus venosus atrial septal defect and the utility of three dimensional echocardiography

364 - Partial anomalous pulmonary venous return in the setting of an inferior sinus venosus atrial septal defect and the utility of three dimensional echocardiography

Does Transcatheter Therapy Replace Surgery? Transcatheter Closure of Sinus Venosus Atrial Septal Defect and Partial Pulmonary Venous Return.

Does Transcatheter Therapy Replace Surgery? Transcatheter Closure of Sinus Venosus Atrial Septal Defect and Partial Pulmonary Venous Return.

Midterm postoperative outcomes of different types of surgical reconstruction of sinus venosus atrial septal defects with anomalous pulmonary venous connection: The Results of Prospective Cohort Study.

Sinus venosus atrial septal defects (SVASDs) constitute a substantial part of atrial septal defects and are usually characterized by anomalous pulmonary venous connection (APVC), causing complications like sinus node dysfunction and arrhythmias. Several surgical approaches are used for treating SVASDs in pediatric patients, including single- and two-patch techniques. The study aimed to prospectively evaluate and compare the safety and efficacy of these two methods with different follow-up periods. Ten patients aged 1-8 years with SVASDs and partial APVC were enrolled in the study at Bhanubhai and Madhuben Patel Cardiac Centre, Karamsad, India, between December 2018 and October2021. The single-patch (sandwich-patch) technique was used in two patients, whereas the two-patch (dual-patch) technique with autologous pericardium was used in seven. Safety was assessed as the absence of complications in the follow-up periods of 6 months, 1, and 2 years, whereas efficacy was estimated by the preserved sinus rhythm and the development of arrhythmias. Electrocardiographic and echocardiographic methods were used to evaluate both parameters. No deaths, reoperations, pulmonary vein, and superior vena cava (SVC) stenosis or phrenic nerve palsy were observed among the 10 patients in the three follow-up periods. Sinus rhythm was arrested in two of the seven patients who underwent two-patch repair, whereas no rhythm disturbances occurred in those who underwent single-patch repair. Both techniques used in SVASD repair with autologous pericardium proved to cause the smaller rate of complications in midterm postsurgical phase. However, there is a potentially great risk of the development of sinus node malfunction after the application of the two-patch technique. Therefore, methods avoiding sinus node interference are preferred in patients with partial APVC involving SVC.