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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.)

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.)

Introduction: In this study, we present a case of directing persistent left superior vena cava drainage into the unroofed coronary sinus to the right atrium in a patient with tetralogy of Fallot and coronary sinus orifice atresia without innominate vein., Case Report: A 16-month-old boy diagnosed with tetralogy of Fallot was admitted. Following intracardiac repair, the left superior vena cava was divided from the left atrial junction, passed through under the ascending aorta and anastomosed to the right superior vena cava., Conclusion: To our knowledge, this is the first patient reported with persistent left superior vena cava, unroofed coronary sinus, coronary sinus orifice atresia, and tetralogy of Fallot. The extracardiac direct anastomosis technique was used successfully to direct persistent left superior vena cava to the right atrium without any post-procedure complications.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism ( P  < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism ( P  = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.

Background : Unroofed coronary sinus is a congenital cardiac anomaly usually associated with persistent left superior vena cava. Premature restriction or closure of foramen ovale is described in association with hypoplastic left heart syndrome. Abdominal peritoneal bands when present manifest clinically. Case report: A 27 years, gravida 2, presented with intrauterine fetal death at 24 weeks gestation due to fetal congestive cardiac failure, cardiomegaly and hydrops. Perinatal autopsy showed absent coronary sinus with cardiac veins draining directly into the heart. There was no persistent left superior vena cava. The foramen ovale was restricted prematurely. The ductus arteriosus was present and non-restrictive. Abdomen showed a cysto-colic peritoneal band. Conclusion : This is the first report showing a triad of (1) complete absence of coronary sinus without left superior vena cava (type-II); (2) premature restriction of foramen ovale without hypoplastic left heart; and (3) a cysto-colic peritoneal band between the gall bladder and colon.

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Exclusive drainage of superior vena cava (SVC) into the left atrium (LA) is one of the rare causes of right-to-left connection. Usually, this anomaly occurs in association with other cardiac defects, and is diagnosed in early childhood. But if it remains an isolated anomaly, this connection may be underdiagnosed and could lead to systemic manifestations. We describe a case of anomalous connection of SVC to LA with drainage of right upper pulmonary vein into SVC and its implications for the perioperative physician. The patient presented with dyspnea on exertion, cyanosis, and a past history of cerebral abscess., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 International Anesthesia Research Society.)

Partial anomalous pulmonary venous connection is a rare congenital abnormality. We present images from an atypical case of isolated anomalous right superior pulmonary venous return to the inferior vena cava without other cardiac abnormalities.

Background: Prenatal diagnosis of persistent left superior vena cava is increasing, but little is known about outcomes of infants with prenatally diagnosed isolated persistent left superior vena cava., Objective: To assess the outcomes of infants with isolated persistent left superior vena cava diagnosed prenatally compared with infants with associated malformations., Methods: All cases of persistent left superior vena cava confirmed by specialized fetal echocardiography in pregnant women were included from a single-centre prospective registry. Unfavourable outcome was defined as termination of pregnancy, in utero death, postnatal death or severe genetic syndrome missed prenatally., Results: A total of 256 infants were included: 113 cases (44.1%) with isolated persistent left superior vena cava and 143 cases (55.9%) with associated malformations; respectively, 111 (98.2%) and 101 (70.6%) had a live birth. The median postnatal clinical follow-up was 3.6 years. Five-year postnatal survival with good outcome was estimated at: 100% (95% confidence interval 90.7% to 100%) in infants with isolated persistent left superior vena cava; 91.0% (74.0% to 98.1%) in infants with associated cardiac anomalies; 87.5% (51.8% to 97.3%) in infants with associated extracardiac anomalies; 81.0% (52.6 to 94.6%) in infants with both cardiac and extracardiac anomalies; and 78.9% (36.7% to 95.9%) in infants with non-structural anomalies. All genetic findings and syndromes were detected in fetuses or infants with non-isolated persistent left superior vena cava., Conclusion: Infants with isolated persistent left superior vena cava have good short-term outcomes postnatally, but persistent left superior vena cava is frequently associated with other malformations that have an effect on outcomes, which should be thoroughly searched for prenatally., (Copyright © 2022. Published by Elsevier Masson SAS.)

Aims: The aim of our study was to investigate the persistent left superior vena cava (PLSVC) cases diagnosed in the prenatal period in our clinic and to compare the obstetric and genetic outcomes of isolated PLSVC cases with cases accompanied by other cardiac or extracardiac anomalies., Methods: The cases diagnosed as PLSVC between January 2015 and January 2019 in our perinatology clinic were evaluated retrospectively. Patients were divided into two subgroups as isolated PLSVC and PLSVC accompanied by another anomaly. Furthermore, patients with extra anomalies were divided into three groups which are cardiac anomaly, extracardiac anomaly and those with both. The groups were compared in terms of genetic results and obstetric outcomes., Results: 89 patients were included in our study. Cases with positive pregnancy outcomes were significantly higher in the isolated PLSVC group than with extra anomaly group (p < 0.001). No karyotype anomaly was observed in the isolated group. Pregnancy results were significantly worse (postpartum demise, termination of pregnancy, in utero demise) in with both cardiac and extracardiac anomalies group (p < 0.001). There was no significant difference between the groups in terms of karyotype results (p = 0.535)., Conclusion: The diagnosis of PLSVC has gained importance and it can be made easier due to the fact that anatomic imaging can be performed in more detail. The isolated PLSVC cases have a very good prognosis. Obstetric outcomes vary according to the accompanying anomaly., Competing Interests: Declaration of Confict of interest All authors declare no conficts of interest., (Copyright © 2022. Published by Elsevier Masson SAS.)

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.

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-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.

Background: The desirable distance, defined as offset, between the central line of the superior vena cava (SVC) and the intra-atrial conduit after an intra-atrial conduit (IAC) Fontan's procedure remained unclear. We compared the hemodynamic features using virtual surgery with different offset designs in our study., Methods: Three-dimensional models of IAC Fontan's procedure were reconstructed according to the magnetic resonance imagings (MRIs) of three patients, then four models for each patient with different offsets equaling 100, 67, 33, and 0% of the diameter of the IVC were reconstructed. Computational fluid dynamics (CFD) were performed in each model to predict the best hemodynamic features, including streamlines of blood flow, wall shear stress (WSS), energy loss (EL), and the hepatic flow distribution (HFD) ratio., Results: Comprehensive evaluation of WSS, EL, and HFD revealed than an offset of 33% presents the best hemodynamic performance among the three patients modeled. In patient A, an offset of 33% resulted in the best HFD (left pulmonary artery/right pulmonary artery [LPA/RPA] = 35/65%). In patient B, the best trade-off between HFD (35/65%), and WSS was achieved with an offset of 33%. In patient C, EL peaked at an offset of 0% and significantly dropped at an offset of 33% with a desirable HFD (60/40%)., Conclusions: We verified that the offset distance influences hemodynamic performance in IAC Fontan's procedure. Considering several hemodynamic parameters, the best trade-offs between hemorheology, pulmonary perfusion, and energy efficiency were achieved at an offset of 33%. This distance should be taken into consideration and optimized during the surgical planning for the IAC Fontan's procedure., Competing Interests: None disclosed., (Georg Thieme Verlag KG Stuttgart · New York.)

Competing Interests: Competing interests: None declared.

Rerouting of persistent left superior vena cava is necessary in correction of certain congenital cardiac conditions. Techniques previously described involve either an "intracardiac" baffle or an "extracardiac" connection between the left superior vena cava and the right atrium or its appendage or the right superior vena cava. We present a modification of the extracardiac technique, utilizing flaps of right and left atrial appendages in construction of the extra-anatomic tube. Using this technique in 7 patients, we obtained a tension-free, oblique anastomosis with vascular tissue circumferentially and potentially preserving growth., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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.)

Background: Persistent left superior vena cava (PLSVC) is one of the most frequently reported congenital anomalies and may be an important source of trigger of atrial fibrillation (AF)., Methods: This was a multicenter retrospective experience including 28 patients with PLSVC who were referred for catheter ablation for drug-refractory symptomatic AF. Pulmonary vein and PLSVC isolation were performed (3.5-mm open irrigated tip ablation catheter at maximum power of 20 W, maximum temperature 43 °C with flow rate of 17 ml/min). Clinical outcomes such as complications and long-term freedom from AF were measured., Results: The mean age of the population was 61 ± 8 years, 21% were females, and AF duration was 60 ± 33 months. Sixty-one percent paroxysmal AF (17/28), 25% (7/28) persistent AF, and 14% (4/28) had long-standing persistent AF. There were no major complications that required any intervention. PLSVC isolation was achieved in 96% (27/28). Freedom from AF at 1 year without antiarrhythmic drugs was seen in 75% (21/28) of patients., Conclusions: In PLSVC patients with AF, segmental isolation of PLSVC appears to be feasible and safe and can translate into favorable clinical outcomes.

Persistent left superior vena cava is the most common congenital anomaly of thoracic venous return, which results when the left anterior cardinal vein fails to regress. A 41-year-old African American male with a history of an unspecified childhood cardiac murmur presented to the emergency department with congestive heart failure exacerbation revealing an incidental finding of a persistent left superior vena cava. Ultimately, he required implantable cardioverter defibrillator placement and cardiac transplantation assessment. In the setting of advanced device placement or cardiac transplantation, a persistent left superior vena cava warrants several important clinical considerations at a center capable of addressing the possibility of a right-sided approach and transplantation irregularities.

Anomalous drainage of the right superior vena cava into the left atrium (LA) has been previously reported in the medical literature. We present a unique case of a child who had two superior vena cavae, a left superior vena cava that drained normally through the coronary sinus to the right atrium, and a right superior vena cava that drained anomalously to the LA.

We report a rare case of persistent left superior vena cava (PLSVC) with direct drainage into the left atrium in a 3-yearsold boy who had been electively admitted for hypospadias repair, when he was noticed to have finger clubbing and mild hypoxia but was otherwise asymptomatic. The diagnosis of PLSVC can be made without an invasive tool as direct drainage of PLSVC into the left atrium be visualised using transthoracic echocardiography (TTE) by injecting agitated saline into the left arm.

Background: Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We describe isolated partial anomalous pulmonary venous return in a 65-year-old woman who was treated by caval division with pericardial patch baffling through a surgically created atrial septal defect and reconstruction of the superior vena cava using a prosthetic graft., Case Presentation: A 65-year-old Asian woman who presented with exertional dyspnea was diagnosed with isolated partial anomalous pulmonary venous return. The surgical indications and strategy were controversial because of the rarity of this pathology. She had an indication for surgery because she was symptomatic and had a high ratio of pulmonary to systemic blood flow. We considered that surgical procedures should avoid postoperative stenosis of a reconstructed flow tract, sinus node dysfunction, and thrombogenesis. We created a caval division with pericardial patch baffling through a surgically created atrial septal defect and reconstructed the superior vena cava using a prosthetic graft for the isolated partial anomalous pulmonary venous return. She has since remained free of exertional dyspnea, arrhythmia, and thrombotic complications. This surgical strategy is safe and effective for treating isolated partial anomalous pulmonary venous return in older symptomatic adults., Conclusions: The long-term outcome of surgical repair of partial anomalous pulmonary venous return with an intact atrial septum in our patient, a symptomatic 65-year-old woman, was excellent.

Purpose: Persistent left superior vena cava (PLSVC) is rare and often asymptomatic, which might cause difficulties in the implantation of cardiac implantable electronic devices (CIEDs). The aim of this study was to determine the prevalence of PLSVC and to study the clinical characteristics and significance of PLSVC in patients with CIED implantation., Methods: Thirty-three patients with PLSVC were collected from 7047 CIED recipients over recent 10 years in our center. These 33 patients were divided into three groups: PLSVC in the absence of RSVC, with a pacemaker implantation (group A, n = 7); double superior vena cava (DSVC), having both PLSVC and RSVC, with a pacemaker or implantable cardioverter defibrillator (ICD) implantation (group B, n = 20); DSVC with a cardiac resynchronization therapy/cardiac resynchronization therapy defibrillator (CRT/CRT-D) implantation (group C, n = 6). The clinical characteristics, pacing parameters, and complications were collected and compared among three groups., Results: The prevalence of PLSVC in our patients implanting CIEDs in the recent 10 years was 0.47%. Coronary sinus diameter, right atrium lead type (active or passive), right atrium lead location (right atrium appendage or lateral wall), right ventricle lead location (right ventricular apex or right ventricular outflow tract), and procedure duration were found to be significantly different among three groups, while the incidence of complications and pacing parameters was not significantly different among three groups during long-term follow-up., Conclusions: Although the presence of PLSVC made the CIED implantation more challenging and might increase the complications related to the procedure, the pacing parameters were not likely to be affected during long-term follow-up.

Single ventricle patients with interrupted inferior vena cava (IVC) and azygos continuation to the superior vena cava (SVC) are typically palliated with a bidirectional cavopulmonary shunt (BCPS), known as the Kawashima operation in this setting. Because the volume of venous blood directed to the pulmonary arteries is substantially greater in the presence of interrupted IVC, Kawashima procedures are commonly delayed to older age compared to other single ventricle patients undergoing BCPS. We report two young infant single ventricle patients with interrupted IVC and azygos continuation to the SVC who underwent stage I Norwood procedures for initial palliation. In both cases, a fenestrated hemi-Fontan procedure achieved successful Kawashima circulations.

We propose a new surgical technique for superior cavopulmonary anastomosis in patients with functionally univentricular heart and bilateral superior caval veins. One of the reasons for failure of bidirectional Glenn shunts in patients with bilateral superior caval veins is the small caliber of one or both veins, with limited flow through each cavopulmonary anastomosis that can easily result in torsion, blockage, or clot formation. The conversion of two small superior caval veins into a single confluence which is large enough to connect with the pulmonary artery (PA) can resolve this problem. We present our experience with two cases in which a rolled pericardial graft was used to create a single caval vein to provide balanced pulmonary blood flow and yield growth of the central PA as well as reducing the likelihood of thrombus formation.

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.

Reports on the incidence of persistent left superior vena cava (PLSVC) in the normal population are limited to studies involving pacemaker implantation candidates and cadavers. The incidence in patients with congenital heart diseases (CHDs) is estimated to be higher than that in the normal population; however, the details are unclear. To investigate the incidence of PLSVC in the normal population and in patients with CHDs, subjects were examined prospectively using echocardiography. Normal subjects consisted of 2841 successive neonates without intra-cardiac or congenital anomalies born in Gifu Prefectural General Medical Center. Additionally, 1920 patients with CHDs were evaluated. The incidence of PLSVC in normal neonates was 0.21% (95% confidence interval 0.042-0.38%). A high incidence (more than 7.0 times the incidence in normal subjects) was observed in all CHD patients. The high incidence group included coarctation of the aorta (CoA) (23.7%) and double outlet right ventricle (DORV) patients (24.6%). The second group consisted of CHD patients with ventricular septal defect (VSD), with an incidence ranging from 5.1 to 6.1%. The low incidence group comprised patients with other CHDs, with an incidence between 1.5 and 3.1%. The incidence of PLSVC in trisomy 21 and atrial septal defect patients was significantly higher than that in normal neonates. The incidence of PLSVC in the normal population and in patients with CHDs was systematically evaluated for the first time. The incidence in CHD patients appeared to be positively influenced by the type of CHD, particularly by DORV, CoA, and VSD.

We herein report a case of atrial tachycardia (AT) originating from the dilated coronary sinus (CS) connected to a persistent left superior vena cava. The earliest activation site of AT was localized at the superior CS ostium, identified using a novel ultra-rapid high-density mapping system (Rhythmia™). Successful ablation was performed at the corresponding position. This report demonstrated the utility of an ultra-rapid high-density mapping system in identifying arrhythmogenic foci in adult cases of congenital heart disease.

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.)

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.

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.)

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.

Over the last decade, the shortage of donors has led to increased waiting time prior to transplantation and its related mortality. Therefore, extended criteria for donor hearts have been proposed. In this report, we describe a successful transplantation despite a diagnosis of partial abnormal pulmonary venous return associated with an atrial septal defect sinus venosus and persisting left-sided superior vena cava. Knowledge in congenital cardiac disease can broaden the definition of 'marginal' donor hearts and allow their use without increasing the risk of transplantation., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.

The incidence of congenital heart disease requiring specialized care is 2.5 to 3 per 1000 live births with a prevalence of congenital heart disease of 81.4 per 10,000 live births. Total cavopulmonary or atriopulmonary connection, used for palliation of certain types of congenital heart disease, diverts flow from the vena cava or atrium directly into the pulmonary arteries. Altered anatomy in patients who have undergone this intervention may result in contrast and/or radiotracer localizing preferentially to a single lung leading to interpretation errors and redundant studies. Performing bilateral upper-extremity injections for this patient population may reduce such technical errors and redundant studies.