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Background: The hypoplastic coronary sinus (CS) is a rare anomaly of the cardiac venous system, wherein some of the tributaries fail to join the CS. These tributaries usually drain into atrial chambers through dilated thebesian channels. We report the first case where the tributaries are draining into the right superior vena cava (SVC)., Case Summary: A case of ischemic cardiomyopathy with severe LV systolic dysfunction with NYHA class III symptoms was taken for CRTD implantation. CS venogram after direct cannulation from left subclavian access revealed a hypoplastic CS. The part of CS beyond the attachment of the oblique vein of the left atrium to CS (distal to the posterolateral vein) formed a common channel and was draining into the right-sided SVC. The posterolateral vein was of sufficient caliber so that an left ventricle (LV) lead could be implanted, and the CRTD procedure could be completed., Discussion: Hypoplastic CS though has no pathological significance in the normal population but for CRT it can become a significant limitation. Tributaries of CS draining into right SVC are the rarest of the finding, the channel draining most likely is a remnant of the splanchnic plexus around the embryonic foregut that usually has a temporary communication with cardinal veins during intrauterine growth. This communication somehow has persisted and resulted in a channel between coronary vein and the SVC, which may be referred to as coronary veno-cardinal vein., (© 2020 Wiley Periodicals LLC.)

Systemic venous anomalies can occur in isolation or in association with other congenital heart disease. A left superior vena cava draining to a roofed coronary sinus represents the most common venous variant and is usually inconsequential. Embryologic or acquired disruptions in systemic veins can be problematic due to shunting and/or venous obstruction. In this case series, we report four systemic venous anomalies in patients with atrial and ventricular situs solitus. Each case demonstrates how clinically significant right to left shunts can occur from a systemic vein to the left atrium. Diagnosis in each of these patients was delayed due to suboptimal diagnostic testing. Accurate diagnosis can be made by thorough anatomic evaluation of the presence, patency, and drainage of all systemic veins and supported with proper planning and interpretation of bubble studies.

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Persistent left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect (Raghib complex) is an uncommon anomaly of systemic venous drainage. We present a unique case of simultaneous presentation of cor triatriatum and persistent left superior vena cava draining into the left atrium in an adult female with partial AV canal and common atrium. Complex intra-atrial baffling including a procedure to redirect flow from a proximal atrial chamber was successful.

The superior vena cava is formed during the fetal period by the development of anastomoses between the right and left anterior cardinal veins, and the regression of the central part of the left anterior cardinal vein. The persistence of this part of the left anterior cardinal vein causes the formation of a left superior vena cava, which is a rare anomaly in cadaver dissection. We report the case of a persistent left superior vena cava with a normal right superior vena cava in a 95-year-old male cadaver, which was discovered during anatomical dissection for medical students at Kawasaki Medical School in 2016. The left superior vena cava was formed by the confluence of the left internal jugular and left subclavian veins and terminated in the right atrium via what would normally be the coronary sinus. The right and left superior venae cavae received intercostal veins via a right and left azygos vein, respectively. However, the right azygos vein was shorter than the normal azygos vein and received only the second to fifth intercostal veins, whereas the left azygos vein received the fifth to eleventh left intercostal veins and the sixth to eleventh right intercostal veins. We consider that the anomalies of the azygos venous system were the result of regression of right supracardinal vein and the persistence of the left supracardinal vein during development. An awareness of such variations of major thoracic veins is important for the interpretation of unusual CT images.

Persistent left-sided superior vena cava (PLSVC) is the commonest systemic venous anomaly in the thorax with a reported prevalence of up to 0.5% in otherwise normal population and up to 10% in patients with congenital heart disease (CHD). In the absence of associated CHD, it is usually asymptomatic, discovered incidentally. It may complicate catheter or pacemaker lead placement. PLSVC typically drains into the right atrium through the coronary sinus. In children with CHD, the presence of a PLSVC may affect the choice of certain surgical procedures. PLSVC is significantly more common in association with situs ambiguous than with situs solitus or inversus, up to 60-70%. In patients with situs ambiguous, the drainage of LSVC is variable, more commonly directly into the atria rather than through the coronary sinus (CS). Rarely, there is a PLSVC draining into the CS with absent right SVC. PLSVC draining into the right atrium via the CS will not usually cause blood shunting between the right and the left sides. However, shunting occurs when PLSVC is associated with unroofed CS, or when it directly drains into the left atrium. With an increased use of CT and MRI for chest and cardiac imaging, PLSVC is being more encountered by radiologists than before. In this article, we will discuss the embryology of PLSVC, its anatomic course and drainage pathways, as well as its clinical relevance and relation to congenital heart disease and viscero-atrial situs.

Left superior vena cava draining into the left atrium in the absence of coronary sinus is an anomaly that can appear in heterotaxy syndrome and unroofed coronary sinus syndrome. Regardless of the origin of these syndromes, biventricular repair can be done through rerouting by intracardiac procedures or through disconnection-reconnection of the left superior vena cava to the right atrium or right superior vena cava by extracardiac procedures. Different techniques can be used for this purpose, each of which has its own advantages and limitations. Therefore, appropriate selection is necessary to obtain the best results for each patient, and many factors, such as patient anatomy, age, associated cardiomyopathies, etc., have to be considered. In this review, we focus on heterotaxy and unroofed coronary sinus syndromes, associated cardiomyopathies, the state-of-the-art in their surgical treatment and our results in a sample of 10 patients. Our experience highlights the importance of accurate diagnosis and specific selection of surgical technique for the management of biventricular repair in patients with left superior vena cava draining into the left atrium in the absence of coronary sinus., (Copyright © 2018. Published by Elsevier Masson SAS.)

We present a 32-year-old patient with cortriatriatum sinister with Raghib's complex (a left persistent superior vena cava draining into the left atrium with an absent coronary sinus and an atrial septal defect [ASD]) who underwent successful surgical correction with excision of the cortriatriatum, closure of the ASD, and establishing the drainage of the persistent left superior vena cava to the right atrium via interposition of an extracardiac 13-mm ringed polytetrafluoroethylene conduit., (© 2017 Wiley Periodicals, Inc.)

Aims The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management. Methods and Results Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug., Conclusions: Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Persistent left superior vena cava (PLSVC) is the most common congenital malformation of thoracic venous return and is present in 0.3 to 0.5% of individuals in the general population. This heart specimen was dissected from a 35-year-old male cadaver whose cause of death was determined as non-cardiac. The heart was examined and we found a PLSVC draining into the coronary sinus. The right superior vena cava was present with a small-diameter ostium. An anomalous pulmonary vein pattern was observed; there was a common trunk to the left superior and left inferior pulmonary veins (diameter 17.8 mm) and an additional middle right pulmonary vein (diameter 2.7 mm) with two classic right pulmonary veins. The PLSVC draining into the coronary sinus had led to its enlargement, which could have altered the cardiac haemodynamics by significantly reducing the size of the left atrium and impeding its outflow via the mitral valve.

Background: Congenital anomalies of the venous system are rare, involve the inferior vena cava (IVC), a persistent left superior vena cava (PLSVC), and the left hepatic vein (LHV), and can make cardiac diagnostic and therapeutic procedures difficult., Case Presentation: We present a 67-year-old woman without heterotaxy syndrome associated with interruption of the left IVC that continued with the hemiazygos vein system, a PLSVC, and an anomalous LHV draining the into coronary sinus (CS). The venous anomalies caused a CS aneurysm. The anomalies were demonstrated by echocardiography and the diagnosis was established by contrast-enhanced computed tomography. Three days later, a coronary artery bypass graft was performed, which confirmed the diagnosis. Half a month after surgery, the pain had been relieved and the patient was discharged from the hospital., Conclusion: Echocardiography is a useful modality to diagnose and assess anomalies of the CS, including CS aneurysms. Congenital anomalies of the venous system in this case were all due to embryonic development abnormalities. Contrast-enhanced computed tomography provides a more comprehensive view of the entire course of abnormal veins.

Persistent left superior vena cava (PLSVC) is a rare congenital anomaly whose prevalence is 0.3 % of general population. The majority of PLSVC drain into right atrium (RA) through the coronary sinus without clinical harm. However, in about 10 % of patients with PLSVC, it drains into left atrium (LA) causing right-to-left shunt. Here, we present a 60-year-old male patient with a PLSVC draining into LA, who developed dyspnea and desaturation depending on the body position after trans-catheter coil embolization of coronary to pulmonary artery fistulas. PLSVC draining into LA should be included in the differential diagnosis of positional desaturation.

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

Background: Repair of unroofed coronary sinus defect in the presence of left superior vena cava is challenging and requires prosthetic graft material to redirect flow to the right atrium. This may potentially cause a supra-mitral gradient or pulmonary venous obstruction., Methods: Three patients with unroofed coronary sinus in the presence of a left superior vena cava (LSVC) underwent modified cavo-atrial anastomosis (Warden technique) to achieve reimplantation of the LSVC in a retro-aortic fashion to the right atrial appendage., Results: Three patients recovered well with no evidence of an intracardiac shunt. Postoperative echocardiography demonstrated normal venous flows in the LSVC., Conclusion: This modified technique offers correction of this systemic venous anomaly without the need for any additional graft material., (© 2016 Wiley Periodicals, Inc.)

We present a case of atresia of the right atrial ostium of the coronary sinus. Recognizing this anomaly is important during catheter ablation or cardiac surgery, which could be noninvasively evaluated by multidetector CT., (Copyright © 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

A 14-year-old male patient presented with cyanosis and tiredness. The patient had undergone a Glenn procedure at age 12 following the echocardiographic determination of a double inlet left ventricle, ventriculoarterial discordance, moderate valvular-subvalvular pulmonary artery stenosis, non-restrictive inlet ventricular septal defect and right ventricle hypoplasia; his oxygen saturation was 70%. Echocardiography evaluation showed retrograde flow from the vena cava superior to the innominate vein and a left superior vena cava (LSVC) opening into the coronary sinus (CS). Here, we report the case of a patient evaluated for Glenn dysfunction in whom an increase in oxygen saturation was observed following transcatheter occlusion of the LSVC-CS connection using an Amplatzer septal occluder.

Atresia of the coronary sinus (ACS) is a rare congenital anomaly. When associated with persistent left superior vena cava (PLSVC), this defect could have no significant hemodynamic effect, and the patient might remain asymptomatic. However, vascular interventions might induce changes or complications that could show the anomaly. Appropriate management requires a good understanding of this condition. We present the first reported case of ACS and PLSVC occurring after thrombosis of the innominate vein (IV) after central venous catheter placement. The patient presented with atypical subacute chest pain and recurrent extrasystoles. Diagnosis and characterization of vascular anomalies was made by computed tomography phlebography, and the patient was successfully managed by endovascular recanalization of the IV.

We report a unique case of a 4-year-old boy with intermediate-type atrioventricular septal defect, cor triatriatum sinister, persistent left superior vena cava, unroofed coronary sinus, and absent right superior vena cava. Persistent left vena cava draining into the unroofed coronary sinus was demonstrated easily using the agitated saline-contrast echocardiography. After conformation with angiographic evaluation, surgery was performed at a single session. Roofing of the coronary sinus with polytetrafluoroethylene patch, mitral cleft repair, tricuspid annuloplasty, atrioventricular defect repair with pericardial patch, and resection of the membrane in the left atrium was succeeded without complication., (© 2013 Wiley Periodicals, Inc.)

Persistence of a left-sided superior vena cava (PLSVC) with absent right superior vena cava (isolated PLSVC) is a very rare venous malformation and commonly associated with congenital heart disease or alterations of the cardiac situs. We describe an unusual case of a young boy presenting with persistent atrial tachycardia and congestive heart failure. He was detected to have unexplained grossly dilated right atrium, right ventricle with systolic dysfunction and a giant coronary sinus (CS). The dilated CS closely mimicked a pseudo cor-triatriatum on echocardiography. Contrast echocardiography from both arms revealed opacification of the CS before the right atrium. Bilateral upper limb venography confirmed the presence of absent right SVC and isolated persistent left SVC draining into the giant coronary sinus., (Copyright © 2013 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

We report a rare case of a 5-year-old child who presented with chronic hypoxemia and a normal cardiac examination, and was found to have a persistent left superior vena cava draining directly into the left atrium. The coronary sinus was absent. This case introduces a contradiction to the generally accepted concept that this anomalous connection can only occur in the presence of a partly or completely unroofed coronary sinus. High index of suspicion is required to include this pathology in the differential diagnosis for a patient with persistent cyanosis with normal cardiac examination.

Unroofed coronary sinus (coronary sinus-type atrial septal defect) is a rare congenital anomaly resulting from imperfect development of left atrio-venous fold. This malformation is not considered suitable for percutaneous treatment due to complex anatomy and risk of coronary sinus obstruction. This article reports on the first successful treatment of partially unroofed coronary sinus by implantation of an Amplatzer(®) Septal Occluder device (AGA Medical Co., Golden Valley, MN). In this patient, the malformation was deemed favorable for device closure due to the local anatomy and the association with persistent left superior vena cava draining in coronary sinus., (Copyright © 2012 Wiley Periodicals, Inc.)

An 11-month-old boy with a functionally single ventricle, coronary sinus ostial atresia, and bilateral superior vena cava (SVC) underwent coronary sinus unroofing upon a right bidirectional cavopulmonary shunt. A persistent left SVC left open to guarantee dual coronary venous drainage became dilated with cephalocaudal blood flow reversal and desaturation. The left SVC was surgically ligated on postoperative day 35.

A persistent left superior vena cava (PLSVC) is formed by the remains of the oblique vein of the left atrium, which is not completely degenerated during embryonic development. The incidence is approximately 0.3% in the general population. Approximately 80-92% of PLSVCs drain into the right atrium through the coronary sinus. This report describes a rare case of PLSVC in which the coronary sinus (CS) did not open into the right atrium, but rather drained into the left subclavian vein through the PLSVC.

This report describes the unique case of a newborn with total anomalous pulmonary venous connection to an unroofed coronary sinus associated with Ullrich-Turner syndrome and aortic coarctation. Total anomalous pulmonary venous connection to the unroofed coronary sinus is an extremely uncommon cardiac abnormality. This congenital heart disease is difficult to diagnose and rarely ever reported. Few symptoms are to be expected when it is not associated with other congenital heart defects. This lesion results in a "naturally" corrected total anomalous pulmonary venous return and a coronary sinus atrial septal defect presenting as a left-to-right shunting lesion. Making the diagnosis of this cardiac defect, and if possible, surgical repair is of importance because cardiac dysfunction due to significant atrial left-to-right shunting is a known long-term complication in the older adult patient.

Cardiac hemangiomas represent 1 to 2% of all detected benign heart tumors. Tumors in the coronary sinus have been reported; however, to our knowledge, there have been no reports of masses in a persistent left superior vena cava. We report here the first case of a 58-year-old man with a rare huge unicamerate cardiac hemangiomas in a persistent left superior vena cava. A communication vein between the coronary sinus and hemangiomas could be identified, and thrombus formation was found in the hemangiomas as well., (Georg Thieme Verlag KG Stuttgart · New York.)

We present a case of an infant with an unroofed coronary sinus associated with a persistent left superior vena cava draining into the left atrium, right superior pulmonary vein stenosis, an atretic left superior pulmonary vein and a double-outlet right ventricle. For pulmonary vein stenosis and atresia, we used a sutureless technique with an autologous pericardial patch to create a neoatrium.

Left superior vena cava (LSVC) draining into the left atrium is a rare congenital cardiac complex. It may appear as an isolated anomaly or as part of more complex cardiac anomalies. Intraatrial rerouting techniques have been the most common approaches to correcting LSVC drainage into the left atrium in patients without a connecting vein. Although these techniques have proved reliable and successful, there are cases in which extracardiac methods for managing this form of anomalous systemic drainage may be preferable. In the present report, we describe an extracardiac approach to the correction of LSVC draining into the left atrium.

Unlabelled: Real-time three-dimensional echocardiography can surpass simple cross-sectional echocardiography in providing precise details of cardiac lesions. For the purpose of optimising treatment, we describe our findings with real-time three-dimensional echocardiography when interrogating different types of communications permitting interatrial shunting. A three-dimensional reconstruction of defects within the oval fossa enabled reliable identification of location, size, and integrity of surrounding rims. In the superior sinus venosus defect associated with partially anomalous pulmonary venous drainage, three-dimensional reconstruction helped to provide a better understanding of the relationship between the interatrial communication, the orifice of the superior caval vein, and the connections of the right upper pulmonary vein. In the defect opening infero-posteriorly within the oval fossa, three-dimensional reconstruction helped to avoid the risk of potentially inappropriate closure of the defect by suturing the hyperplastic Eustachian valve to the atrial wall, which could have diverted the inferior caval venous return into the left atrium, or obstructed the caval venous orifice. In the coronary sinus defect, three-dimensional echocardiography provided a 'face to face' view of the entire coronary sinus roof, showing a circular defect communicating with the cavity of the left atrium. Acquisition of the full-volume data sets took less than 2 minutes for the patients having defects within the oval fossa, and no more than 3 minutes for the patients with the sinus venosus and coronary sinus defects. Post-processing for the defects in the oval fossa took from 5 to 8 minutes, and from 12 to 16 minutes for the more complicated defects., Conclusion: Cross-sectional two-dimensional echocardiography can establish correct diagnosis in all types of atrial communications; however, real-time three-dimensional reconstruction provides additional value to the surgeon and interventionist for better understanding of spatial intracardiac morphology.

Persistent left superior vena cava is known to be associated with cor triatriatum. The persistent left superior vena cava drains into a dilated coronary sinus and can cause left atrial outflow obstruction by impinging on the posterior wall of the left atrium. Residual obstruction can persist, even after surgical resection of the cor triatriatum membrane. We describe a novel surgical treatment in a patient with cor triatriatum and persistent left superior vena cava.

This report describes the preoperative diagnosis of a 6-month-old child with tricuspid atresia related to an unroofed coronary sinus. The anomaly usually is diagnosed after the Fontan operation due to persistent arterial desaturation. Preoperative recognition of this association is important. All cases of tricuspid atresia should be screened for associated persistent left superior vena cava draining into an unroofed coronary sinus.

We report a case of an unusual congenital triad consisting of absence of coronary sinus, persistent left superior vena cava and scimitar syndrome incidentally found in a CT-scan performed on a female complaining of exertional dyspnea.

In the modern implanting era with progressive expanding indications to resynchronization therapy, upgrading procedure is a relatively common event. Persistent left superior vena cava (PLSVC), the most common venous abnormality, may exacerbate technical difficulties. We describe the procedure of upgrading from a dual chamber pacemaker to resynchronization/defibrillation system with a total of 4 leads through a PLSVC entering a dilated coronary sinus (CS) never described before. The case report, in addition to the description of a unique technical approach, raises a lot of clinical questions about how many leads we can introduce in such a venous structure and inside CS without hemodynamic impact on venous drainage potentially leading to life-threatening situations., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Fetal valproate syndrome (FVS) results from prenatal exposure to valproic acid. It is characterized by a distinctive facial appearance, a cluster of minor and major anomalies and central nervous system dysfunction. We describe a 2-month-old male infant with the typical dysmorphic features characteristic of FVS. He had a persistent left superior vena cava draining into a dilated coronary sinus and mild pulmonary hypertension. There was a history of maternal intake of sodium valproate during pregnancy.

A 41-year-old man with tetralogy of Fallot and dextrocardia, coronary sinus atrial septal defect and partially unroofed coronary sinus with persistent left superior vena cava (PLSVC) underwent a total correction of these anomalies. An association between these specific anomalies has not been reported previously in the literature. These defects were successfully corrected by a) closure of the coronary sinus atrial septal defect with PLSVC entering the right atrium using a pericardial graft; and b) ventricular partition with a Dacron patch and conotruncal repair for tetralogy of Fallot. The postoperative course of this patient has been uneventful.