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Superior sinus venosus defects (SVD) are interatrial communications located above the confines of the oval fossa, where unroofing of the right upper pulmonary vein leads to its anomalous drainage to the superior venacava. Recent emergence of transcatheter closure of these defects using covered stents is an attractive alternative option especially in adults with additional comorbidities. This article focuses on various aspects of non-surgical closure of SVD, including patient selection, appropriate hardware options, step-by-step procedural details, evolution and modifications in the techniques over the last decade, protocols for follow-up evaluation, and potential complications associated with this intervention., Competing Interests: Disclosure The author has nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Atrial fibrillation is the most common sustained cardiac arrhythmia in adults and it is associated with a high burden of mortality and morbidity worldwide. Catheter ablation is increasingly used to improve symptoms and prognosis in selected patients. Lower limb venous access with subsequent transseptal approach to the left atrium is the standard procedure for atrial fibrillation catheter ablation., Case Presentation: We report an unusual case of complex venous anomaly with a left-sided inferior vena cava with hemiazygos continuation to a persistent left superior vena cava draining in an enlarged coronary sinus in a patient with persistent atrial fibrillation scheduled for transcatheter ablation., Discussion: Lower limb venous anomalies may limit a standard transseptal approach to the left atrium thus precluding an effective catheter ablation procedure for atrial fibrillation. Alternative interventions, such as unconventional percutaneous access, thoracoscopic approach and "ablate and pace" procedures, may be necessary in patients with symptomatic atrial fibrillation and complex venous anomalies., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

In an adult patient, coronary artery fistula involving the circumflex artery (CX) connected to the coronary sinus caused aneurysm of the left main and CX associated with ectasia of the intermediate branch. The patient had posterolateral infarction with severe ischemic mitral regurgitation and moderate tricuspid regurgitation. A rare venous return anomaly was also present. The inferior vena cava, which was interrupted at the level of the liver, continued with the hemiazygos to drain into a persistent left superior vena cava, which in turn drained into the coronary sinus. Surgery included CX closure and mitral and tricuspid repair. The strategy had to be adapted to the anatomy. The fistula was dissected and snared for cardioplegia delivery, and venous return was achieved by cannulation of the superior vena cava and femoral vein. The procedure was uneventful, and 4 years later the patient is asymptomatic., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this article., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Children undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction., Methods: A retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021. Simple SVC anatomy included a single right SVC to the right atrium or bilateral SVCs with a left SVC to an intact coronary sinus, without prior superior cavopulmonary connection. Presence of anomalous SVC anatomy, superior cavopulmonary connection, or previous atrial switch operation defined complex anatomy. Reconstructive strategies included atrial anastomosis; direct SVC-to-SVC anastomosis; and augmented SVC anastomosis using innominate vein, patch, cavopulmonary connection, or interposition graft. The primary outcome was reintervention for SVC obstruction., Results: Of 288 patients, pretransplant diagnoses included congenital heart disease (n = 155 [54%]), cardiomyopathy (n = 125 [43%]), and other (n = 8 [3%]). Most (n = 208 [72%]) had simple SVC anatomy compared with complex SVC anatomy (80 [28%]). Reintervention for SVC obstruction occurred in 15 of 80 (19%) with complex anatomy and 1 of 208 (0.5%) with simple anatomy (P = .0001). Reintervention was more common when innominate vein or a patch was used (9/25 [36%]) compared with an interposition graft (1/7 [14%]) or direct anastomosis (6/82 [7%]; χ 2  = 13.1; P = .001). Most reinterventions occurred within 30 days of OHT (14/16 [88%])., Conclusions: Patients with complex SVC anatomy have a higher rate of reintervention for SVC obstruction after OHT compared with those with simple SVC anatomy. In cases of complex SVC anatomy, interposition grafts may be associated with less reintervention compared with complex reconstructions using donor tissue., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 10-month-old female spayed Scottish Fold was referred to cardiology for incidental radiographic cardiomegaly. Echocardiography was suspicious for a right atrial or right auricular aneurysm. The differential diagnosis also included peritoneal-pericardial diaphragmatic hernia, mass lesion (cyst, granuloma, or neoplasia), or cardiac malformation. A giant right atrial aneurysm associated with a persistent left cranial vena cava was subsequently confirmed with computed tomography., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Partial anomalous pulmonary venous return with intact atrial septum warrants greater understanding and evaluation in the literature., Methods: From January 1993 to December 2018, 293 patients with partial anomalous pulmonary venous return underwent surgical repair. Of these, 45 patients (15.3%) had an intact atrial septum. The median age was 36 years (interquartile range, 24-48). Direct reimplantation was used in 17 patients (38%), intracardiac baffling in 15 (33%), and caval division (Warden) technique in 13 (29%). Descriptive statistics were used to assess the data, and Kaplan-Meier analysis was used to assess survival., Results: Anomalous veins were right-sided in 27 patients (60%), left-sided in 16 patients (36%), and bilateral in 2 patients (4%). The insertion sites were the superior vena cava in 23 patients (51%), innominate vein in 12 (27%), inferior vena cava in 6 (13%), coronary sinus in 2 (4%), right atrium in 1 patient (2%), and unknown in 1 patient (2%). Scimitar syndrome was noted in 8 patients (18%). There was no postoperative mortality or residual defects. Postoperative echocardiography excluded any obstruction of pulmonary or systemic veins. Postoperative complications included atrial fibrillation in 9 patients (20%) and pneumothorax requiring chest tube in 5 patients (11%). Survival at 1, 5, and 10 years was 100%, 95%, and 95%, respectively. Two patients underwent pulmonary vein dilation, one at 3 years and the other at 7 years., Conclusions: Surgical repair of partial anomalous pulmonary venous connection with intact atrial septum can be performed with excellent early and midterm outcomes. The overall incidence of midterm systemic or pulmonary vein stenosis is low., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Operative repair of partial anomalous pulmonary venous connection (PAPVC) remains challenging due to risks of sinus node dysfunction, baffle obstruction, and superior vena cava (SVC) obstruction., Methods: Traditional or modified Warden procedures were performed in 75 of 318 consecutive patients (24%) with PAPVC repaired surgically at our institution during 1993 to 2021. Clinical characteristics, echocardiography data, operative details, and early and late outcomes were collected. Cumulative incidence of reintervention and Kaplan-Meier survival analysis are reported., Results: Median age was 39 years (interquartile range, 21-57 years). Fifty-nine (79%) had normal sinus rhythm preoperatively. Seventeen (23%) had intact atrial septa. Traditional and modified Warden procedures were performed in 15 (20%) and 60 (80%), respectively. Frequent concomitant procedures included 15 (20%) tricuspid valve repairs and 12 (16%) atrial fibrillation procedures. There were no early deaths. Postoperative complications included atrial fibrillation in 17 (23%), sinus node dysfunction in 15 (20%), pneumothorax in 3 (4%), pleural effusion in 2 (3%), and pacemaker implantation in 1 (1%). At hospital discharge, sinus node dysfunction persisted in 8 (11%). Over a median follow-up of 6 years (interquartile range, 4-10 years), baffle obstruction developed in 1 patient and SVC obstruction developed in 7. None required reoperation and 6 were treated with SVC stents. At 1, 5, and 10 years, the cumulative incidence of reintervention was 5%, 7%, and 14%, and survival was 99%, 94%, and 94%, respectively., Conclusions: Traditional and modified Warden procedures can be performed with satisfactory early and late survival. Persistent sinus node dysfunction and need for permanent pacing are low. Late SVC obstruction is uncommon and can often be managed nonoperatively., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Sinus venosus defect (SVD) is an unusual type of interatrial communication (IAC) and is virtually always associated with partial anomalous pulmonary venous drainage (PAPVD) of the right pulmonary veins (RPV) to the superior vena cava (SVC) or right atrium (RA). However, its definite morphogenesis is still elusive, and diagnostic fallibility continues., Methods: We conducted a retrospective review of the echocardiograms, cardiac catheterization data, computed tomographic findings, and surgical notes of 44 children with surgery-confirmed isolated SVD from 1977 to 2016. We investigated the location of the IAC and its boundaries within the atrial septum and its anatomic relationship with the adjacent structures, including the anomalously draining RPV. We also tried to explore any possible associated abnormalities which might be implicated in the morphogenesis of SVD., Results: Two distinct types of IAC were defined. Forty patients had an IAC that was located posterosuperior to the intact fossa ovalis (superior type), and all were associated with PAPVD of the right upper and often the right middle pulmonary veins to the SVC. The remaining 4 patients had an IAC that was located posterior to the intact fossa ovalis (inferior type), and all were associated with PAPVD of all the RPV to the RA. Another consistently associated abnormality was a defect between the anomalously draining RPV posteriorly and the SVC or RA anteriorly. All these 44 patients underwent successful surgical baffling the associated PAPVD via the IAC into the left atrium., Conclusion: A defect between the RPV posteriorly and the SVC or RA anteriorly will result in SVD, and an unusual type of IAC, and PAPVD of the RPV to the SVC or RA. The IAC is not a true atrial septal defect in the atrial septum proper, but it actually represents the left atrial orifice of the unroofed RPV., (Copyright © 2019. Published by Elsevier B.V.)

A 75-year-old woman was referred to our hospital for dyspnea and edema of the lower limbs. Computed tomography angiography revealed a dilated left coronary artery from the left main trunk to the left circumflex branch and a dilated fistula originating from the left circumflex branch and draining into the persistent left superior vena cava. Physical examination revealed pulmonary hypertension and congestive heart failure. We performed closure of the fistula and left main coronary artery orifice and coronary artery bypass grafting. Postoperative computed tomography angiography showed patency of all grafts and progression of thrombosis in the dilated abnormal vessels., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Bilateral absence of the superior vena cava (SVC) is an uncommon congenital vascular anomaly, mainly asymptomatic, usually undetected, and often associated with other cardiac anomalies. Though usually harmless and totally innocent, this vascular anomaly might complicate cardiovascular surgery, the insertion of a central venous catheter and the transvenous placement of a pacemaker. This SVC anomaly is still not well known, underdiagnosed and its incidence is much higher than described. A better understanding of this anomaly and its detection could play a key role in avoiding its potential complications. We are sharing a case of a female adult, with no medical history, who presented herself to the department of visceral surgery with a collateral venous circulation of the upper thorax, that was at first, mistaken for a portal hypertension syndrome, findings were pushed to finally conclude a bilateral absence of the SVC., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: The electrophysiological features and roles of persistent left superior vena cava (PLSVC) in supraventricular tachycardia (SVT) are not known., Objective: The purpose of this study was to elucidate the electrophysiological features and roles of PLSVC in patients with SVT., Methods: We included 37 patients with PLSVC (mean age 43.5 ± 17.1 years; 35.1% men) and 510 patients without PLSVC (mean age 43.9 ± 18.8 years; 48.2% men) who underwent an electrophysiology study for SVT. The number of induced tachycardias, location of the slow pathway (SP) or accessory pathway (AP), and radiofrequency catheter ablation (RFCA) outcomes were compared between patients with and without PLSVC. During RFCA of the left AP, a coronary sinus (CS) catheter was placed into the left superior vena cava (left superior vena cava group) or the great cardiac vein (great cardiac vein group). The RFCA outcomes were compared between the groups., Results: In patients with PLSVC, 40 tachycardias were induced: atrioventricular nodal reentrant tachycardia (AVNRT) (n = 19), atrioventricular reentrant tachycardia (n = 17), and focal atrial tachycardia (n = 4). Among patients with AVNRT, an SP in the CS was significantly more frequent in patients with PLSVC than in those without PLSVC (47.4% vs 3.8%; P < .001). In patients with the left AP, the number of RFCA attempts and recurrence were lower in the great cardiac vein group than in the left superior vena cava group., Conclusion: An SP in the CS is prevalent in patients with AVNRT and PLSVC. It is useful to place a CS catheter into the great cardiac vein in patients with a left AP and PLSVC., (Copyright © 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Orthotopic heart transplantation in patients with an isolated persistent left superior vena cava is extremely rare, and the anastomotic connection between a right-sided donor superior vena cava and left-sided recipient superior vena cava can be challenging to perform. We present a novel technique used in an infant female, using the left atrial appendage to extend the superior vena cava anastomosis., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The double-switch operation for congenitally corrected transposition of the great arteries, in which the morphologic left ventricle is restored to the systemic circulation, is an effective surgical option. This case report describes an atrial switch technique without using supplemental material during the double-switch operation for congenitally corrected transposition of the great arteries associated with persistent left superior vena cava, with the systemic venous blood flow rerouted through the enlarged coronary sinus., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report a novel method of constructing an extracardiac bidirectional inferior cavopulmonary anastomosis. Our patient was a 4-month-old girl with complex single-ventricle anatomy and a small-caliber superior vena cava. A direct anastomosis from the inferior vena cava to the main pulmonary artery was constructed by using all autologous tissue. The resulting pathway remained patent and exhibited growth with age. Furthermore, a pulmonary arteriovenous malformation did not develop. This case demonstrates how a bidirectional inferior cavopulmonary anastomosis is feasible in the human and can provide similar hemodynamic results as the bidirectional superior cavopulmonary anastomosis., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A persistent left superior vena cava (SVC) results from failed obliteration of the left common cardinal vein during embryogenesis, with a spectrum of anatomic variants. We report a rare case of bilateral SVCs connected by a bridging vein and with a direct left SVC connection to the left atrial appendage in an asymptomatic patient without hypoxemia or associated congenital heart disease on transthoracic echocardiography, computed tomography, and magnetic resonance imaging. A multimodality imaging approach is valuable to search for associated anomalies and to confirm this anatomic variant, which has important implications on vascular procedures and avoidance of systemic embolism., (Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

We report the case of a 72-year-old woman who underwent urgent coronary bypass grafting (CABG). Intraoperatively a persistent left superior vena cava (PLSVC) draining into the left atrium was detected. Because of the resulting right-to-left shunt volume even after complete drainage and collapse of the right atrium, the heart was still ejecting in the absence of significant aortic insufficiency. Thorough examination revealed a PLSVC draining into the left atrium. After ligation of the PLSVC, the CABG could be performed as planned., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The Senning and Mustard baffles remain important techniques for the treatment of congenitally corrected transposition (cc-TGA), isolated ventricular inversion, and D-transposition of the great arteries with delayed presentation. We describe the treatment of an 8-month old infant with atrioventricular discordance, ventriculoarterial concordance, and dextrocardia with atrial situs inversus. A modified Senning procedure was performed through the "left-sided" atrium. Modifications of the Senning and Mustard baffles remain important tools in the treatment of rare conditions like isolated ventricular inversion., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The treatment of some subsets of patients having both right-sided superior vena cava (SVC) and left-sided SVC may be very challenging. We performed the SVC integration (SVCI) technique with end-to-side anastomoses between the two SVCs on 4 such patients (age, 5 to 11 months; body weight, 5.4 to 10.2 kg) with excellent outcomes. In 2 patients, we performed intrapulmonary artery septation with SVCI; in 1 patient, pulmonary artery sling repair with SVCI; and in 1 patient with supramitral stenosis related to persistent left-sided SVC, repeated Blalock-Taussig shunt (BTS) with SVCI. Our SVCI technique is very useful in specific cases (eg, in candidates for the Fontan procedure., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Coronary venous anatomy is little studied, and an abnormal coronary vein drainage is a rarely reported entity. Few cases about abnormal drainage of the great cardiac vein into the superior vena cava have been reported in literature. Herein we present a case of anomalous small cardiac vein draining into superior vena cava., (Copyright © 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Background: A persistent left-sided superior vena cava (PLSVC) is a rare, often asymptomatic, venous anomaly that may be first diagnosed during central venous catheterization. During chest radiograph interpretation, a PLSVC can be confused with inadvertent arterial catheterization., Case Report: We describe the presentation of a 45-year-old man with end-stage liver disease who required central venous catheterization for treatment of septic shock. An aberrantly placed catheter noted on chest radiograph and an elevated central venous oxygen saturation gave the appearance of inadvertent carotid artery catheterization. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: A persistent left-sided superior vena cava can masquerade as an inadvertent cannulation of the carotid artery during central line placement. It is important for emergency physicians to be aware of this possibility when evaluating a chest radiograph with an aberrantly placed catheter. Venous hyperoxia may further complicate attempts to differentiate between arterial and venous catheterization in patients with septic shock. After confirmatory tests, the emergency physician should consider removal of the catheter due to potential complications., (Copyright © 2014 Elsevier Inc. All rights reserved.)

A 5-month-old female French bulldog was evaluated for the presence of a heart murmur. Through clinical and echocardiographic evaluations, a severe Type A pulmonary stenosis was diagnosed. Angiography during right ventricular catheterization for valvuloplasty revealed drainage from a persistent left cranial vena cava (PLCVC) into the left atrium; this was confirmed later by contrast echocardiography. This report is the first to describe this anatomical variant of a PLCVC in a dog., (Copyright © 2014 Elsevier B.V. All rights reserved.)

The term nutcracker syndrome refers to compression of left renal vein between aorta and superior mesenteric artery causing renal venous hypertension. Right nutcracker syndrome associated with a left-sided inferior vena cava is an extremely rare anomaly. Reported two cases in English literature were diagnosed by ultrasonography and computed tomography angiography in adulthood. Herein, we present a case of right nutcracker syndrome with left-sided inferior vena cava and hemiazygos continuation in a 12-year-old girl., (Copyright © 2014 Elsevier Inc. All rights reserved.)

In patients with partial anomalous pulmonary venous return of the right superior pulmonary veins to the superior vena cava, surgical repair generally consists of either intraatrial baffle with or without caval enlargement, or superior caval transection and cavoatrial anastomosis to the right atrial appendage. We discuss here a novel technique of superior caval enlargement without need for patch material or reimplantation., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)