Surgical Correction of a Sinus Venosus Atrial Septal Defect with Partial Anomalous Pulmonary Venous Connections Using Cardiac Computed Tomography Imaging and a 3D-Printed Model.

Sinus venosus atrial septal defects (SVASDs), concurrent with partial anomalous pulmonary venous connections (PAPVCs), are a rare congenital heart disease in dogs. Surgical correction is essential when clinical signs or significant hemodynamic changes are present. We aimed to report on the successful surgical correction of an SVASD with PAPVCs, using a computed tomography (CT)-based customized 3D cardiac model. A 10-month-old male poodle was referred for corrective surgery for an ASD. Echocardiography confirmed a hemodynamically significant left-to-right shunting flow through an interatrial septal defect and severe right-sided heart volume overload. For a comprehensive diagnosis, a CT scan was performed, which confirmed an SVASD with PAPVCs. A customized 3D cardiac model was used for preoperative decision-making and surgical rehearsal. The defect was repaired using an autologous pericardial patch under a cardiopulmonary bypass (CPB). Temporary pacing was applied for sinus bradycardia and third-degree atrioventricular block. The patient recovered from the anesthesia without further complications. The pacemaker was removed during hospitalization and the patient was discharged without complications 2 weeks post-surgery. At the three-month follow-up, there was no shunting flow in the interatrial septum and the right-sided volume overload had been resolved. The cardiac medications were discontinued, and there were no complications. This report indicates the validity of surgical correction under CPB for an SVASD with PAPVCs, and the advantages of utilizing a CT-based 3D cardiac model for preoperative planning to increase the surgical success rate.

Cor Triatriatum Dexter With a Sinus Venosus Atrial Septal Defect in a 50-Year-Old Woman: A Case Report.

The diagnosis of atrial septal defect (ASD) may be delayed until adulthood or even later in life as it is a well-tolerated congenital heart disease. If patients are not examined and investigated well in childhood, the diagnosis may be delayed until later in adulthood when patients present with palpitations and sometimes dyspnea due to the right chambers dilatation from right ventricular volume overload. In this report, we present a case of a 50-year-old female patient with symptoms of heart failure and atrial fibrillation who was found to have dilated right cardiac chambers, dilated pulmonary artery, severe tricuspid regurgitation, pulmonary hypertension, and a pulmonary-to-systemic flow ratio (Qp/Qs) of more than 1.5 by transthoracic echocardiography and Doppler, indicating left to right shunt at the atrial level. However, transthoracic echocardiography could not visualize the defect, and two-dimensional (2D) transesophageal echocardiography was done in this patient and documented the presence of a sinus venosus ASD with an incomplete cor triatriatum dexter membrane; all four pulmonary veins were identified going to the left atrium. Since the presence of an incomplete cor triatriatum dexter membrane (despite causing no symptoms) makes the percutaneous closure of the sinus venosus ASD and the percutaneous repair of tricuspid regurgitation very difficult, we decided to advise surgical ASD closure and tricuspid valve repair for the patient.

A Case of Sinus Venosus Atrial Septal Defect: Physical Examination as a Diagnostic Clue.

An atrial septal defect (ASD) may be detected later in life due to its asymptomatic status. We report a case of superior sinus venosus ASD, a rare type of ASD, in which bedside physical examination was useful for the diagnosis. A 72-year-old male was referred to cardiology during the treatment of a cerebral infarction. On examination, a right ventricular heave, a split-second heart sound with an increased pulmonary component, and a systolic ejection murmur in the pulmonary region were noted. Transthoracic echocardiography showed a systolic pulmonary artery pressure of 50 mmHg with right heart enlargement, but there was no shunt flow. Because an agitated saline contrast study was positive, transesophageal echocardiography was performed and demonstrated direct flow between the left atrium and superior vena cava. Our report highlights the importance of considering ASD, such as sinus venosus type, even in the absence of transthoracic echocardiographic findings suggestive of this condition, when patients present with a bedside physical examination consistent with ASD.

Long-term outcome after surgical correction of sinus venosus defect in a nationwide register-based cohort study.

OBJECTIVES: Long-term results after sinus venosus defect (SVD) closure are sparse and many studies lack a proper control cohort. This nationwide cohort evaluated the long-term outcome after SVD surgery. METHODS: The study enrolled every surgical SVD correction from the nationwide hospital discharge registry (FHDR) and surgical registries of two tertiary centers. Patients with more complex congenital heart defects were excluded. Surgeries were performed from 1969 to 2019. Five sex and birth-year-matched controls per SVD patient were gathered from the general population. RESULTS: In total, 182 surgical SVD corrections were performed during the study period. The median age at the time of surgery was 8.3 years (range 0.06-75.7), and the majority (77.5%, n = 141) were under 18 years old. The median follow-up period was 18 years (range 0.1-53). There was no significant difference in mortality during the follow-up (logrank p = 0.62, MRR 0.78, 95% CI: 0.30-2.0). However, SVD patients had elevated risk for new-onset atrial fibrillation (RR 4.9, 95% CI: 2.2-10.9), heart failure (RR 4.0, 95% CI: 1.2-13.2), ischemic heart disease (4.3, 95% CI, 1.5-11.7), migraine (RR 3.6, 95% CI: 1.5-9.1) and sick sinus syndrome, II- or III-degree AV-block or pacemaker implantation (RR 11.3, 95% CI: 2.9-43.8). CONCLUSION: Young patients with SVD have an excellent survival prognosis after the surgery. Risk for sick sinus syndrome or conduction disorders, atrial fibrillation, and heart failure remains elevated in the long-term follow-up.

Transcatheter correction of sinus venosus defect in a patient with a challenging anatomical configuration: From bench testing to clinical success.

We report successful transcatheter correction of a sinus venosus defect in a 72-year-old woman with anomalous pulmonary venous return in a challenging anatomical configuration. The procedure was facilitated by hands-on simulation training on a newly developed, perfused, 3D-printed model.

Transcatheter Closure of Superior Sinus Venosus Defects.

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

Atrial Fibrillation Ablation in a Patient With SV ASD and PAPVR Preceding Transcatheter Septal Closure.

Atrial fibrillation (AF) is common in adults with unrepaired atrial septal defects (ASDs). Sinus venosus (SV) ASDs associated with partial anomalous pulmonary venous return (PAPVR) are traditionally managed surgically. We report the first AF catheter ablation in a patient with SV ASD and PAPVR preceding transcatheter ASD repair with a covered stent. (Level of Difficulty: Advanced.).

A rare case of sinus venosus atrial septal defect: Malpositioned right atrial pacemaker lead in the left atrium.

Incidental discovery of sinus venosus atrial septal defect (SV-ASD) in the elderly is rare. This defect allows for lead malpositioning during pacemaker placement and can lead to catastrophic cardioembolic events. Post-pacemaker implantation, chest radiography should be obtained to detect malpositioning early, and if detected, lead adjustment is recommended; if identified later, treatment with an anticoagulant is feasible. SV-ASD repair may be considered as well.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: SV-ASD with the APVs draining high to the SVC could be repaired safely and effectively through port access with a double-patch technique.

Superior caval vein partially overriding the interatrial septum-A fetal echocardiographic sign of superior sinus venosus defect. Reporting its rare association with coarctation of aorta in a fetus and its outcome.

Prenatal diagnosis of superior sinus venosus defect has never been reported in the literature. I describe here an echocardiographic sign which is helpful in diagnosing this defect in a 35 + 2 weeks fetus and its rare association with coarctation of aorta.

Modified approach for transcatheter correction of superior sinus venosus atrial septal defect: a case series.

Background: Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous drainage (PAPVD) of the right upper and/or right middle pulmonary vein (RUPV/RMPV) has recently been described as an alternative to surgical approach in a substantial number of patients. We describe a modified technical approach for transcatheter correction of SVASD using transoesophageal echocardiography (TOE) to confirm adequate stent landing zone and apposition and evaluate its feasibility, safety, and procedural success. Case summary: From 2019 to 2021, three consecutive patients received a transcatheter correction of SVASD with PAPVD by redirecting the superior vena cava and RUPV/RMPV to the left atrium by implantation of a custom-made covered Cheatham platinum stent (10-zig, length: 60-80 mm). Prior to stent implantation, a balloon testing was performed in the anticipated landing zone using TOE to confirm complete defect closure and unobstructed pulmonary venous drainage. Stent deployment and flaring of the interior stent portion were performed with TOE guidance to confirm adequate landing zone and apposition and to avoid residual shunt or pulmonary vein obstruction. Conclusion: Transcatheter correction of SVASD with PAPVD was performed without any complications. The follow-up period was 7.8, 13.6, and 29.8 months, respectively. During follow-up, no mortality, stent embolization, or obstruction of pulmonary venous drainage occurred. The TOE-guided modified transcatheter approach for correction of SVASD with PAPVD is safe and feasible with excellent post-procedural results and represents an alternative to surgical treatment in a pre-selected patient cohort.

Surgical Treatment of a Defect Recurring 22 Years after Closure of an Inferior Sinus Venosus Defect: A Case Report.

BACKGROUND: Residual shunt after closure of an inferior sinus venosus defect (ISVD) is a rare complication with a high rate of reintervention. CASE PRESENTATION: Here, we report a rare case of a recurrent defect identified 22 years after closure of an ISVD. The defect (25 × 10 mm) was located at the inferior vena cava-right atrial junction and was closed directly when the patient was 5 years of age. No residual shunt was detected and follow-up was discontinued at age 12 years. However, a residual atrial septal defect shunt was detected incidentally at age 27 years. During the second surgery, the lower end of the original defect was opened and then closed with an expanded polytetrafluoroethylene patch. CONCLUSIONS: Because of the high rate of reintervention for residual shunt after ISVD closure, patch closure was selected as a better option to reduce tension at the inferior-posterior border. Patients with this profile should be followed closely, at least during childhood, including by echocardiography.

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.

Background and Aims: 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. Methods: 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 October 2021. 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. Results: 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. Conclusion: 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.

A new FDA approved stent for congenital heart disease: First-in-man experiences with G-ARMORTM.

We present the first clinical experience with a new hybrid cell structure covered stent, designed for congenital heart disease applications. It represents a significant redesign of the Cheatham Platinum (CP) Stent (Numed Inc.), maintaining the traditional benefits of the covered CP whilst significantly decreasing shortening and allowing controlled flaring at the ends through its combination of larger and standard sized cells. We first implanted the stent in 2 patients with superior sinus venosus defects with anomalous drainage of the right upper and middle lobe pulmonary veins. The first was a 40 year male and the second a 36 year old female. The third case was a 60 year old patient with near atresia of the aorta, with pre and poststenotic aortic dilation. The clinical result in all cases was excellent with no obstruction to pulmonary venous return and no visible L-R shunt on the transthoracic echo on 24 h and 2 week follow-up for the patient with sinus venosus defects and uniform complete revascularization of the aorta without any vascular complications in the patient with coarctation. These are the first uses of this stent in human subjects. The design is specifically aimed toward procedures where stent shortening is undesirable. Hence, coarctation of the aorta as well as stent implantation in preparation for percutaneous pulmonary valve placement are obvious use areas, as well as the growing body of evidence supporting percutaneous treatment of sinus venosus defects.

Minimally invasive versus sternotomy approach for double-patch repair of partial anomalous pulmonary venous connection and sinus venosus defect in pediatric and adult patients: Mid to long-term outcomes.

BACKGROUND: Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients. METHODS: This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction. RESULTS: This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm. CONCLUSIONS: The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.

A Patient with Dyspnea and an Enlarged Right Ventricle.

Case report of a patient with no significant past medical history who presented with reports of dizziness, dyspnea on exertion, and palpitations that had been ongoing for at least 5 years. It demonstrates the importance of considering the presence of an inter-atrial shunt when evaluating a patient with an unexplained dilated right atrium and right ventricle.

Absence of atrial smooth muscle in the heart of the loggerhead sea turtle (Caretta caretta): a re-evaluation of its role in diving physiology.

Contraction of atrial smooth muscle in the hearts of semi-aquatic emydid turtles regulates ventricular filling, and it has been proposed that it could regulate stroke volume during characteristic rapid transitions in cardiac output associated with diving. For this hypothesis to be supported, atrial smooth muscle should be widely distributed in diving Testudines. To further understand the putative function and evolutionary significance of endocardial smooth muscle in Testudines, we studied the hearts of loggerhead sea turtles, Caretta caretta (n=7), using immunohistochemistry and histology. Surprisingly, we found no evidence of prominent atrial smooth muscle in C. caretta. However, smooth muscle was readily identified in the sinus venosus. Our results suggest that atrial smooth muscle does not contribute to the diving capabilities of C. caretta, indicating that the possible roles of smooth muscle in emydid turtle hearts require a re-evaluation. In sea turtles, the sinus venosus may instead contribute to regulate cardiac filling.

Descending thoracic aortic homograft for superior vena caval translocation in the Warden procedure: Report of six cases.

BACKGROUND: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. METHODS: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. RESULTS: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. CONCLUSIONS: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.

Novel technique for transcatheter closure of sinus venosus atrial septal defect: The temporary suture-holding technique.

BACKGROUND: Transcatheter repair of sinus venosus atrial septal defect (SVASD) has become an alternative option to surgical repair. There are potential significant complications related to stent stability in the superior vena cava (SVC) and potential migration of the stent that need to be addressed. Therefore, the technique is still evolving. OBJECTIVES: To report results of a new modification "the suture technique" that improves safety profile of positioning and securing a covered stent in the SVC. METHODS: This is a descriptive, single center, retrospective review of patients who underwent SVASD closure using the suture technique at our institution between 02/2020 and 08/2022. RESULTS: Fourteen patients underwent transcatheter repair of SVASD using the suture technique. All procedures were successful. The suture technique allowed precise stent placement in all patients without any migration or complication. Six patients required additional stent placement at the level of the SVC. One patient had an additional covered stent placed to eliminate a tiny residual shunt. Two patients had negligible residual shunts at the time of the procedure. At follow-up, all patients clinically improved and had significant reduction in right heart size on echocardiography and/or magnetic resonance imaging. No arrhythmia was reported in any patient. None required re-intervention after a mean follow-up of 16.5 ± SD 10.5 months. CONCLUSIONS: The suture technique appears to be safe modification. Although our study involves small sample size with no comparative group, we believe our technique offers greater control over stent positioning, reducing the risk of stent embolization and residual shunting in transcatheter closure of SVASD.

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

Background: Transcatheter 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. Methods: From 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. Results: 3DP 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. Conclusions: Assisting 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.