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Persistent left superior vena cava (PLSVC) draining to the left atrium (LA) is a rare congenital abnormality that is often asymptomatic and found incidentally on imaging. PLSVC is usually described alongside other congenital defects, such as septal defects, tetralogy of fallot, and aortic coarctation. PLSVC to LA with an atrial septal defect is known as Raghib syndrome, but to our knowledge PLSVC to LA without an atrial septal defect or right superior vena cava has not been described in the literature. Here, we report the presentation of a patient with PLSVC-LA without ASD and propose a classification system for this subset of congenital heart defects to help guide clinical and surgical management of these patients., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This study aims to define the associated anomalies with PLSVC, and to compare single PLSVC and bilateral superior vena cava in terms of accompanying anomalies and pregnancy outcomes. This was a retrospective study of the fetuses diagnosed with single and/or bilateral SVC at a tertiary fetal medicine center during 8 years. We detected 16 cases of single PLSVC and 84 cases of bilateral SVC. We found an association between the PLSVC and cardiac and extracardiac anomalies. Comparison between single PLSVC and BSVC cases revealed significant differences in the occurrence of heterotaxy and right isomerism. The study highlights the importance of prenatal diagnosis in PLSVC cases. Isolated PLSVC with situs solitus may be considered a benign finding, but larger studies are needed to understand the clinical implications of PLSVC in relation to chromosomal anomalies. Routine screening protocols should include three-vessel and trachea views to detect PLSVC., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Coronary anomalies encompass different conditions in terms of anomalous origin, coursing, and branching, which are occasionally associated with congenital heart disease. This study aimed to explore coronary anomalies associated with ventricular septal defect that was a stereotypical congenital heart disease. We retrospectively reviewed angiographic findings in patients with ventricular septal defect who required corrective surgery, and identified coronary abnormalities, including anomalous origin, coursing, and branching of coronary arteries based on angiography. We studied the prevalence and types of coronary anomalies among them. A total of 998 patients with ventricular septal defect was studied. Age and weight were 2.0 (2.1-15.2) months and 5.7 (4.1-8.7) kg, respectively. There were 115 patients (12%) with syndrome or genetic disorder, and 34 patients (3%) with extracardiac major organ anomalies. The overall prevalence of coronary anomalies was 6.2%. All coronary anomalies were anomalous origin and coursing, among 3 patients accompanied anomalous intrinsic coronary anatomy. Originating within aortic root above the Valsalva sinus was the most common coronary anomaly. Coronary anomalies were independently correlated with bicuspid aortic valve (odds ratio [OR]: 8.02, 95% confidence interval [CI]: 2.34-23.4) and persistent left superior caval vein (OR: 5.02, 95% CI: 1.93-11.7). We showed the possibility that minor cardiac variants, such as bicuspid aortic valve and persistent left superior caval vein, contribute to higher prevalence of coronary anomalies in patients with ventricular septal defect., (© 2022. Springer Japan KK, part of Springer Nature.)

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.)

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Partial anomalous pulmonary venous drainage (PAPVD) is a common congenital heart disease. If the insertion of an anomalous pulmonary vein (PV) is high into the superior vena cava (SVC), the traditional 1-patch or 2-patch surgical repair might be challenging. Unlike patch procedures, the cavoatrial anastomosis technique (Warden procedure) theoretically reduces complications such as sinus node dysfunction and venous obstruction. We investigate outcomes of the Warden procedure (WP) at a single institution. A total of 67 patients (42 males and 25 females) with PAPVD who underwent the WP between January 2011 and December 2018 were consecutively enrolled. The median age was 2.8 years (3-61 years old). 52 cases were younger than 18 years old. perioperative and follow-up clinical data were collected. In addition, we selected possible risk factors (a total of 18 risk factors) of SVC stenosis or obstruction. Univariate and multivariate analyses were conducted to confirm the independent risk factors. The average Cardiopulmonary bypass (CPB) time was 132.3 ± 46.3 min, and the aortic cross-clamp time was 85.2 ± 35.7 min. One patient died postoperatively in hospital. The mean follow-up time of the remaining patients (n = 66) was 15.8 ± 14.5 months (3-64 months). No abnormal cardiac function or sinus node dysfunction cases were identified during the follow-up. Of the survival patients, no venous obstruction was presented before the discharge. Numbers of patients identified with mild PV and SVC stenosis were 1 (1.5%) and 7 (10.6%, 4 of them turned negative eventually) at discharge. At the last follow-up, no PV obstruction was identified, whereas, 4 cases (6%) had SVC obstruction. Numbers of PV and SVC stenosis cases were 3 (4.5%) and 8 (12%). Univariate and multivariate analyses showed that only the diameter of SVC less than 10 mm was an independent risk factor for SVC stenosis or obstruction at the last follow-up. Warden procedure can effectively treat PAPVD (anomalous drainages into SVC), with satisfactory early and mid-term postoperative results. Different modifications of the Warden procedure may be selected according to the anatomic characteristics. The diameter of SVC less than 10 mm predicts SVC stenosis or obstruction after Warden procedure.

Risk of superior vena cava (SVC) obstruction following repair of anomalous right upper pulmonary veins in children is unclear. The incidence and outcome of subclinical obstruction remained unknown. Retrospective single institutional study (07/1993-02/2017) in a pediatric population (N = 42, median age 3.9-year, range 0.1-15.3 years). 33 (79%) children had repair without SVC translocation ("non-Warden") and 9 (21%) had Warden-type surgery. Echocardiographic SVC obstruction was defined as (I) turbulent flow across SVC and (II) continuous flow pattern without return to baseline velocity (0 m/s); severe obstruction was defined as loss of distinct biphasic profile ± mean gradient ≥ 5 mmHg. 3 (7%) patients required intra-operative revision due to obstruction (non-Warden: 1, Warden: 2). After discharge, 2 (5%) patients required reintervention (3 and 6-month post-op) for severe symptomatic obstruction (non-Warden: 1, Warden-type: 1). Both patients responded to balloon angioplasty with symptomatic resolution (one required repeat catheter reintervention). 10 (24%) patients had subclinical echocardiographic obstruction (2, 22% Warden vs. 8, 24% non-Warden; p = 1.0; 8 of 10 patients had mild gradient), which resolved and remained well without reintervention. At follow-up (mean 7.2-year, range 0-23 years), all patients were alive. Freedom from SVC reintervention at 10 and 20-year is 95% (97% at 10, 20-year in non-Warden and 89% at 5, 8-year in Warden-type group; log-rank p = 0.34). Surgical repair for anomalous right upper pulmonary veins is associated with risk of SVC obstruction in children. The need for reintervention for severe obstruction is rare at late follow-up. Patients with subclinical obstruction remain asymptomatic and demonstrate echocardiographic improvement.

A persistent left superior vena cava (PLSVC) is a congenital venous abnormality and is usually asymptomatic and does not cause hemodynamic disturbances. Therefore, it is difficult to identify it by routine examinations in clinical practice. This study aimed to elucidate the electrocardiographic characteristics for the prediction of a PLSVC. Twelve patients (9 males, 56.2 ± 18.3 years) who were diagnosed with a PLSVC were enrolled. The electrocardiographic parameters, including the P-wave duration, axis, and morphology of the P waves, were automatically measured and compared to 150 controls (77 males, 57.3 ± 14.6 years). There were no significant differences in the P-wave duration. Negative or positive/negative P waves in lead III predicted a PLSVC with a sensitivity of 100% and specificity of 81%. The P-wave axis in PLSVC exhibited a significant leftward deviation as compared to the controls (14.8 ± 21.1 vs. 54.0 ± 17.4°, p < 0.001). A receiver operating characteristic curve analysis of the P-wave axis for predicting a PLSVC exhibited an area under curve of 0.93 [CI 95% (0.87-0.98), p < 0.001), and identified a P-wave axis of less than 37.5° to have a 92% sensitivity and 83% specificity in predicting a PLSVC. A negative or positive/negative P-wave morphology in lead III was a useful finding for suggesting the presence of a PLSVC.

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.

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.

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.

A 37-year-old patient, who suffered from a repeated superior vena cava (SVC) syndrome, was scheduled for stent implantation into SVC, but suffered from contrast anaphylaxis. To monitor the procedure, we used intra-cardiac echocardiography and successfully implanted a stent. Placing an intra-cardiac echocardiographic catheter in the main pulmonary artery and facing towards the right, we could readily visualize stenosis in the SVC and inflation of the stent. Also looking up from right atrium, we noted proximal obstruction of the stent and confirmed the relief of obstruction after additional balloon dilation. This report leads to new application of intra-cardiac echocardiography for intervention of structural and vascular diseases other than inter-atrial septum.

Cor triatriatum is a rare cardiac anomaly sometimes associated with tetralogy of Fallot (TOF) and persistent left superior vena cava (PLSVC). We present the case of a 2-year-old girl who was diagnosed with a unique form of cor triatriatum that was associated with TOF and PLSVC. In this case, the abnormal membrane that arose from the posterior wall of the left atrium encircled the left superior vena cava (LSVC) in the left atrial cavity. Here, we discuss this rare case as well as the imaging studies and surgical strategy adopted.

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).

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 the case of successful endovascular treatment of large saccular aneurysm of SVC in a patient with vascular malformation of right hand and chest. Considering the high risk of surgery, the patient was referred for percutaneous intervention. Venography showed communication between the aneurysm and SVC, just below brachiocephalic confluence. That is why the decision of balloon-protected transcatheter thrombin injection was made. Selective catheter was placed in the aneurysm and balloon occlusion catheter in SVC. Both catheters were withdrawn right after thrombin injection. During follow-up, aneurysm slightly enlarged in early observation and after a year shrinkage was observed.

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.

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.

The absence of a right superior vena cava (SVC) in situs solitus is very rare, and even then it is usually associated with left SVC. There have been few case reports in the literature of isolated bilateral absence of SVC. Bilateral absent SVC can have clinical implication, including pacemaker placements, central venous line placement, monitoring, and occasionally associated anomalies. We describe a case detected on fetal echocardiogram with bilateral absent SVC and structurally normal heart.

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.

Lutembacher syndrome involving the association of congenital atrial septal defect (ASD), usually of the ostium secundum variety, and mitral valve disease is a well-known entity. Its association with a coronary sinus, ASD, and a persistent left superior vena cava (LSVC) draining into the left atrium (LA) (Raghib syndrome) is rarely described in the literature. This association in a 15-year-old boy erroneously deemed to be inoperable before referral to the authors' hospital due to cyanosis in the presence of atrial septal defect (ASD) and mitral stenosis is described in this report. Evaluation by echocardiography followed by cine angiography confirmed the cause of cyanosis to be drainage of the LSVC into the LA together with an ASD and rheumatic mitral stenosis, a combination of Raghib and Lutembacher syndromes. The boy underwent successful surgical correction. The authors believe this is the second such case to be reported in the English literature and the first of its kind to be managed by surgical intervention.

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.

Total anomalous systemic venous drainage is a rare form of congenital heart disease. All the systemic venous flow from the body (i.e., from the superior vena cava, inferior vena cava, and coronary sinus) drains abnormally into the left atrium. This condition requires the presence of a left-to-right shunt (atrial septal defect, patent ductus arteriosus, or ventricular septal defect) to allow the systemic venous return to reach the pulmonary circulation. This disorder may be associated with heterotaxy syndromes. This report describes a patient with unique total anomalous systemic venous drainage that was successfully corrected surgically.

Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure. This unusual fistulous connection has only rarely been reported in the literature, and in those reports, it has not resulted in neonatal heart failure.

We report the case of a patient with symptomatic heart failure referred after an unsuccessful attempt at cardiac resynchronization therapy. An occlusive Thebesian valve prevented entry into the coronary sinus ostium. Careful analysis of the patient's cardiovascular physiology and anatomy revealed the "fortuitous" presence of a persistent left superior vena cava. Cannulation of this vessel permitted percutaneous retrograde placement of a left ventricular lead into a posterolateral cardiac venous branch resulting in successful cardiac resynchronization. This unique case provides strong evidence that thorough knowledge of cardiac embryology, anatomy, and physiology plays a pivotal role in percutaneous electromechanical intervention for drug-refractory heart failure.

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.

Congenital arteriovenous malformations between the subclavian artery and the superior vena cava (SVC) is a very rare anomaly scantily described in the literature. We describe this anomaly in a 4-month-old infant who presented with congestive heart failure. He was diagnosed to have a right subclavian artery to SVC fistula associated with SVC stenosis. The child successfully underwent device (vascular plug) closure of the fistula and SVC stenting. Six months postprocedure, child is doing well, with complete resolution of symptoms.

Scimitar syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.

We present an infant with unusual anatomy and physiology of a persistent left superior vena cava (LSVC). The adverse physiologic consequences of the LSVC were corrected with percutaneous placement of vascular plugs.

A 57-year-old man presented with symptomatic right-to-left shunts decades after surgical closure of an atrial septal defect and partial anomalous pulmonary veins. Two sources of shunting were identified: a baffle leak from the right superior vena cava (RSVC) to the left atrium secondary to complete occlusion of the RSVC-to-right atrium connection and an acquired previously unpublished coronary sinus to the left atrial shunt secondary to inadvertent closure of the coronary sinus. The diagnosis and transcatheter management of these unusual right-to-left shunts are reported.

Patients with respiratory and/or cardiac failure occasionally require the use of extracorporeal membrane oxygenation (ECMO), which presents an obstacle for standard imaging modalities. Computed tomographic angiography (CTA) can be used in patients with congenital heart disease, usually to define extra-cardiac anatomy in the chest. We describe the use of CTA to evaluate two infants with congenital heart disease while on ECMO. The first infant had totally anomalous pulmonary venous connection, and the second had a pulmonary sling that resulted in tracheal stenosis. The studies achieved high diagnostic quality with minimal radiation exposure. Subsequently, both infants had successful surgical repairs.

Right superior vena cava (SVC) drainage into the left atrium causing hypoxemia is a very rare congenital malformation. This anomaly can result in complications of chronic cyanosis and paradoxical embolism. It rarely is associated with any other congenital malformation. This is the first case report of right SVC drainage into the left atrium associated with a coronary cameral fistula. During surgical repair, the aneurysmal part of the cameral fistula was noted to be occluding a defect in the atrial septum. Repair of the cameral fistula and rerouting of the SVC via the atrial septal defect were performed.

Persistence of a left superior vena cava (LSVC) has been reported in 0.3%-0.4% of candidates for pacemaker (PM) or cardioverter-defibrillator (ICD) implantation. The aim of the study was to evaluate the clinical implications of LSVC persistence for proper device performance. We observed the prevalence of LSVC during a 15-year period. A total of 2077 consecutive patients underwent PM implantation over a 15-year period: 7 had persistent LSVCs (0.34%). Among 599 patients undergoing ICD implantation, 4 LSVCs (0.66%) were observed. Overall LSVC persistence was found in 11/2676 (0.41%) patients. The right superior vena cava was absent in 4/11 (36%) patients. The leads were placed from the left subclavian approach in 5/7 PM patients: 2 received an elective right sided approach due to physician preference. All ICD patients had the device placed left pectoral with a single-coil lead: defibrillation therapy was effective in the long term in all but one patient, who required the addition of a subcutaneous array. Left superior vena cava persistence in PM/ICD patients is similar to the general population (0.41% in our study). The left-sided implant may be skill-demanding during lead placement; however, this task can be accomplished in the majority of cases, with a reliable outcome in the short term and appropriate device performance at follow-up.

Kabuki syndrome is a rare congenital disorder characterized by mental retardation, postnatal dwarfism, visceral anomalies, and peculiar facies (resembling the makeup of actors in Kabuki, traditional Japanese theater) initially described in Japan. Congenital heart and great vessel defects are the most frequent visceral anomalies found in children with Kabuki syndrome. Although many cardiovascular defects have been described, a double aortic arch has never been reported with Kabuki syndrome.

We experienced a successful pediatric surgical case of partial anomalous pulmonary venous connection to the superior vena cava with cor triatriatum. Echocardiography and multidetector-row computed tomography showed partial anomalous pulmonary venous connection (right upper pulmonary vein connected to the high superior vena cava) and atypical cor triatriatum (analogue to type III-A2 of Lucas-Schmidt classification: left upper pulmonary vein had dual connection to the innominate vein via vertical vein and the accessory chamber). At 8 years of age, the male patient underwent extracardiac right atrial pedicle repair of partial anomalous pulmonary venous connection to the superior vena cava (Williams' modification) and excision of the diaphragm between the accessory chamber and the left atrium simultaneously. The postoperative course was uneventful in normal sinus rhythm and there was no stenosis of newer drainage root from right upper pulmonary vein.

Cardiac resynchronization therapy (CRT) by biventricular pacing reduces symptoms and improves left ventricular function in many patients with heart failure due to left ventricular systolic dysfunction and cardiac dyssynchrony. Implantation of the biventricular pacing lead in association with persistent left superior vena cava is technically challenging. We report a successful case of minimally invasive video-assisted thoracoscopic left ventricular epicardial lead implantation for biventricular pacing in a patient with persistent left superior vena cava.

We report the first known case of a retro-aortic innominate vein providing continuity between both the left and right atria in the presence of a left superior vena cava and a normal coronary sinus. This unusual structure created a bidirectional shunt with clinical desaturation at baseline and it complicated the management of a 9-year-old patient while undergoing mechanical ventilation. An embryologic mechanism that explains the development of this complex remains elusive.

We describe the unusual case of a 71-year-old male with a history of deep vein thrombosis and recurrent multiple pulmonary embolism (PE) despite adequate anticoagulation. Computed tomography (CT) and brachiocephalic venography revealed a left-sided superior vena cava. We describe successful placement of an inferior vena cava filter via a left-sided superior vena cava.

Absent right superior vena cava with persistent left superior vena cava in normal situs is an exceedingly rare congenital anomaly. In such cases, pacemaker implantation (PMI) is very difficult or even impossible. We report the case of a patient with sick sinus syndrome in whom PMI was easily performed via the left superior vena cava by using a steerable stylet.

Absence of the right superior vena cava (SVC) is a rare event occurring in patients without congenital cardiovascular abnormalities. Since absence of the right SVC is usually clinically silent, its diagnosis is mandatory prior to invasive medical or surgical procedures. We report two cases of echocardiographic diagnosis of absence of the right SVC with persistent left SVC and a large coronary sinus in structurally normal heart in a fetus of 20 weeks' gestation and in a newborn. The diagnosis was confirmed by transthoracic contrast echocardiography with intravenous injection of agitated saline into the right arm.

The unique anatomy, physiology, and surgical repair of a patient with features of three rare congenital heart defects (total anomalous pulmonary venous return to the coronary sinus, cor triatriatum, and unroofed coronary sinus with persistent left superior vena cava to the left atrium) is described. Analysis of this case suggests that these three conditions are linked in that they all may result as a consequence of a defect in the same embryologic process (i.e., incorporation of the pulmonary venous confluence into the left atrium).

We describe an autopsied specimen, from an infant who died in 1967, in which concordant ventriculoarterial connections coexisted with parallel arterial trunks, usually described as "anatomically corrected malposition." Based on their shape, the extent of the pectinate muscles, and the absence of a coronary sinus, we diagnosed isomeric right atrial appendages. Despite this, the arrangement of the remaining thoracic and abdominal organs, as well as the patterns of venous drainage, was almost completely mirror imaged.

A previously healthy 12-year-old girl presented with severe headache for 2 weeks. On physical examination, there was finger clubbing without apparent cyanosis. Neurological examination revealed only papiledema without focal neurologic signs. Cerebral magnetic resonance imaging showed the characteristic features of brain abscess in the left frontal lobe. Cardiologic workup to exclude a right-to-left shunt showed an abnormality of the systemic venous drainage: presence of isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect. This anomaly is rare, because only a few other cases have been reported.

We report an unusual anomaly of the upper body venous drainage in a patient with visceroatrial situs inversus. The patient had a persistent right superior vena cava draining into the left-sided right atrium by way of the coronary sinus, with the absence of the left superior vena cava associated with atrioventricular discordant levocardia, pulmonary atresia, and ventricular septal defect.