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Persistent left superior vena cava (PLSVC) is the most common venous anomaly with an incidence of 0.3-0.5% in the general population. Here, we report a rare case of PLSVC with anomalous atrium in a cadaver during the student's dissection session at the University of Tsukuba. In this case, the coronary sinus had merged with the right atrium to form an enlarged sac-like structure and received systemic venous flow including inflow from the PLSVC. The roof of the coronary sinus with the right atrium was thicker than that of the control cases. We further found that the distance between the sinoatrial node and the opening of the coronary sinus was slightly more than half of that in control cases. This variant appears interesting and is worth reporting for developmental and clinical consideration., (© 2021. Japanese Association of Anatomists.)

A newborn with situs solitus, normally related great arteries and intact atrial septum, underwent surgical repair at our institution for anomalous drainage of the right superior vena cava in the left atrium at the level of the right superior pulmonary veins. This rare cyanotic congenital cardiac malformation is herein described with special regard to its anatomical and diagnostic features. A novel surgical approach for achieving correction is also described.

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.

The present perspective is a synthesis of published investigations in the setting of anomalous connection of the right superior caval vein to the morphologically left atrium or biatrial drainage of the right caval vein. We identified 57 suitable cases from 97 investigations, reviewing the clinical presentation, diagnostic modalities utilized, surgical techniques used, and their outcomes. Clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, radionuclide perfusion scan, magnetic resonance imaging, and angiocardiography provided the diagnostic information and were used to define the disease entities before surgery. We have also addressed several issues concerning the influence of the so-called heterotaxy: the establishment of the diagnosis, the variation in clinical presentation, and subsequent management. For the overall group of patients undergoing either surgical intervention or transcatheter treatment with an Amplatzer vascular plug, the operative mortality remains high at 9.5%. We submit that an increased appreciation of these disease entities will contribute to improved future surgical management.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Although the occurrence of bilateral superior caval veins (SCVs) is not unusual, persistence of the left SCV with atretic right SCV is extremely uncommon in the setting of normal visceroatrial arrangement. We report such a case that was also associated with anomalous pulmonary venous connection of the right pulmonary veins to the solitary left SCV.

Anomalous superior vena cava drainage is a rare congenital cardiac defect where the right superior vena cava anomalously drains into the left atrium causing a right to left shunt. We present a case of a 17-day-old male who presented with cyanosis without respiratory distress and was found to have a right superior vena cava draining anomalously into the left atrium.

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.

Competing Interests: Competing interests: None declared.

Persistent left superior vena cava (PLSVC) is the most common anomalous thoracic venous drainage. A PLSVC usually drains into the right atrium through a dilated coronary sinus. It is rare that a PLSVC flows directly into the left atrium, and even rarer that it connects to the left upper pulmonary vein (LUPV). We report a case, wherein the LUPV connected to both the PLSVC and the left atrium.

Objective: Conventional procedures for partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) still have serious complications, such as late SVC and/or pulmonary venous obstruction and supraventricular arrhythmia. We aimed to introduce our newly developed surgical technique with minimum right atriotomy and double-barreled arrangement of systemic and pulmonary venous channels (double-decker technique)., Methods: From 1998 to 2018, 21 consecutive patients with PAPVR to the SVC underwent this new procedure. The patients' median age and body weight were 4.4 years and 16.5 kg, respectively. Two female patients underwent lateral thoracotomy. Postoperative hemodynamics of both venous channels were assessed using time-resolved 3-dimensional magnetic resonance phase contrast imaging in 6 patients., Results: The median follow-up period was 11.0 years. There was no early mortality and late death. No patient required reoperation and/or intervention. All patients maintained normal sinus rhythm, and supraventricular arrhythmia did not occur. The median blood flow velocity of the neo-SVC and neopulmonary venous channel was 0.40 and 0.30 m/s, respectively. Using time-resolved 3-dimensional magnetic resonance phase contrast imaging, the straight and nonrestrictive flow and low wall shear stress were visualized in both venous channels., Conclusions: Our newly developed double-decker technique is a useful alternative surgical procedure for PAPVR to the SVC. Late complications can be completely avoided using this method. Growth potential of both channels is also maintained., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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.)

Background: Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described., Case Presentation: We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully., Conclusions: The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

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.)

Background: Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT., Methods: In all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015. All of them were evaluated clinically and underwent contrast-enhanced CT., Results: In one patient, the Amplatzer® Septal Occluder was used. In two patients, the Amplatzer® Vascular Plug type-1 was preferred: the device size/LSVC diameter ratio was 1.7 in the child and 1.2 in the adult. There were no early-onset or long-term onset complications. CT was performed 1, 2, and 10 years after the procedure, respectively. Complete occlusion of the vessel was documented in all. After 10 years since the procedure, CT revealed a persistent trivial residual shunt through the accessory hemiazygos vein in one patient, in whom the device was implanted above its drainage into the left superior caval vein. When an Amplatzer® Vascular Plug type-1 is oversized compared with the venous vessel diameter, it immediately assumes a dog-bone shape that disappears early to regain its shape memory and nominal size., Conclusions: Percutaneous occlusion of left superior caval vein draining into the left atrium has excellent early and long-term outcomes. The optimal implantation of the device is below the drainage of the accessory hemiazygos vein, when present. The device might be oversized compared with the left superior caval vein diameter according to the age of the patient.

We report the prenatal diagnosis and the neonatal follow-up of a patient with isolated total abnormal systemic venous connection to the left atrium. Right-sided and left-sided superior caval veins and the inferior caval vein were all connected to the left atrium. Pulmonary venous return was normal. This was associated with some right ventricular underdevelopment. To our knowledge, this is the first fetal description of this very rare congenital cardiac malformation.

We present a case of anomalous direct connection of the right superior caval vein to the left atrium. The diagnosis was made in a 2-year-old child with unexplained cyanosis and apparently normal heart. Corrective surgery was performed, and the child recovered completely.

A 45-year-old man with dyspnoea and palpitations exhibited a unique systemic-to-pulmonary veno-venous connection on preoperative CT images. A window of 31.5-mm diameter was evident between the superior caval vein and the middle pulmonary vein, which was normally connected to the left atrium via a 30-mm-diameter orifice. The atrial septum was intact.

Isolated right superior caval vein drainage into the left atrium is a rare congenital cardiac anomaly usually presenting with hypoxaemia and cyanosis. Surgical repair is the definitive treatment for this condition. In this report, we present the case of a 72-year-old patient with uncorrected anomalous drainage of a right superior caval vein into the left atrium, first diagnosed in the 1960s. To the best of our knowledge, this is the oldest reported unrepaired case.

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.

Biatrial drainage of the right superior vena cava resulting from a sinus venosus defect is a rare congenital malformation. It can result in severe desaturation although a sinus venosus defect usually presents as a left-to-right shunt. A male baby was noted to have cyanosis while nursing and was referred to us for medical treatment on his second day of life. Echocardiography showed that most of the blood flowing through the superior vena cava drained into the left atrium. He underwent successful surgery to correct this defect at the age of 2 years.

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.)

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.

We report the multidisciplinary consideration in a case of right superior vena cava draining into the left atrial roof. A 12-year-old boy was diagnosed with a sinus venosus-type atrial septal defect associated with anomalous right upper pulmonary venous return. However, intraoperative inspection showed the right superior vena cava draining into the left atrial roof, associated with anomalous right upper pulmonary venous return. An anatomical radical operation was performed using a modified conventional technique., (© The Author(s) 2014.)

There are different surgical methods for the repair of persistent left superior vena cava that connects directly to the left atrium. We describe an extracardiac surgical technique that includes direct anastomosis of persistent left superior vena cava to the right atrium with right atrial flap and autologous pericardium. We have performed this procedure in four cases and there is no obstruction at postoperative control studies. Right atrial flap repair is a feasible extracardiac technique that offers growth potential., (Georg Thieme Verlag KG Stuttgart · New York.)

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

A simple total anomalous pulmonary venous connection (TAPVC) coexisting with a persistent left superior vena cava (PLSVC) is extremely rare. Connection of the PLSVC with the coronary sinus behind the left atrium induces coronary sinus dilatation. This reduces the free posterior wall space to which the common pulmonary vein is anastomosed for repairing the anomalous connection. Postoperative recurrent pulmonary venous obstruction (PVO) is the most important complication. To prevent PVO, sufficient tension-free anastomosis is necessary. When dilated, the coronary sinus becomes an obstacle for obtaining sufficient incision length in the left atrial cavity. We encountered two cases of a simple TAPVC with a PLSVC in infants weighing 1.8 and 2.9 kg, respectively. To obtain sufficient incision length, we extended the incision line to the right atrium for an atypical supracardiac TAPVC and incised from the left atrium to the coronary sinus via the right atrium for an infracardiac TAPVC. Moreover, we recreated the atrial septum with a rightward shift using a tanned pericardium in both cases. The postoperative courses were uneventful, without recurrent PVO., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. 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.)

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.)

A 5-year-old girl presented to an ambulatory cardiology clinic with shortness of breath, described by the patient's mother and chest discomfort on exertion. At this visit, she was noted to have low baseline room air oxygen saturations of 89%. Subsequent cardiac, respiratory and haematological investigations including contrast echocardiography, CT scan of the chest, polysomnography and haemoglobin electrophoresis were unhelpful in yielding a diagnosis. A CT scan of the chest with contrast was performed which revealed a persistent left-sided superior vena cava that appeared to be draining into the left atrium, creating a right-to-left shunt. This was not revealed on contrast echocardiography as contrast (micro bubbles) had been injected into the right arm. This case report highlights the nuances of diagnostic investigations, which without a high index of suspicion may exclude a timely diagnosis and therapeutic intervention., (2014 BMJ Publishing Group Ltd.)

Cortriatriatum with Raghib's complex is a rarely reported entity. An 18-month-old baby who presented with tachypnea and cyanosis was diagnosed to have cortriatriatum sinistrum along with a persistant left superior caval vein draining to the left atrium through an unroofed coronary sinus. The child underwent successful surgical correction with excision of the cortriatriatum and baffling of the left superior caval vein to the right atrium.