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The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition., (© 2023 Wiley Periodicals LLC.)

Background: Partial anomalous pulmonary venous connection (PAPVC) occurs when at least one pulmonary vein drains into the right atrium or its tributaries rather than the left atrium, most commonly connecting with the superior vena cava (SVC). The Warden procedure involves transecting the SVC proximal to the uppermost connection of the pulmonary vein followed by proximal SVC reattachment to the right atrial appendage. However, descending thoracic aortic homograft replacement for SVC translocation has recently been introduced as a modified technique., Aims: This commentary aims to discuss the recent study by Said et al. who reported their experiences with six PAPVC cases undergoing a modified Warden procedure using thoracic aortic homograft SVC translocation., Methods: A comprehensive literature search was performed using multiple electronic databases to collate the relevant research evidence., Results: The Warden procedure is associated with a 10% incidence of SVC obstruction with many requiring reintervention. Meanwhile, using the aortic homograft for SVC translocation, Said et al. observed no SVC obstructions. In addition, this modified technique does not require anticoagulation and has demonstrated an improvement in long-term SVC patency. Nevertheless, it can be considered an expensive procedure. Moreover, since the thoracic aortic homograft utilized is biological tissue, only long-term follow-up will determine whether calcification and graft degeneration is an issue., Conclusion: It can be concluded that the modified Warden procedure is a safe and effective method to reconstruct the systemic venous drainage into the right atrium when a direct anastomosis under tension might be prone to re-stenosis., (© 2022 Wiley Periodicals LLC.)

Background: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification., Methods: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022., Results: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways., Conclusions: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation., (© 2022 Wiley Periodicals LLC.)

Background and Aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages., Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts., Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%., Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival., (© 2022 Wiley Periodicals LLC.)

Vein of Galen malformation (VGM) is a rare intracranial vascular malformation. High output heart failure is common in infancy and is characterized by dilatation of all the cardiac chambers. We report an unusual case of VGM in a 3-month-old infant without dilatation of the right-sided cardiac chambers. We then demonstrate importance of comprehensive evaluation in detecting rare coexistence of anomalous right superior caval vein connection to the left atrium., (© 2018 Wiley Periodicals, Inc.)

Right atrioventricular valve is historically named as tricuspid valve. However, cases depicting multi-leaflet morphology of the right atrioventricular valve were reported. Accessory leaflet of the tricuspid valve is a rare entity and associated with congenital cardiac anomalies. We present an accessory tricuspid valve in a patient with isolated persistent left superior vena cava., (© 2018 Wiley Periodicals, Inc.)

Background and Aims: Surgical repair of right partial pulmonary anomalous connection to the superior vena cava (SVC) with the Warden procedure can be complicated by SVC obstruction, pulmonary veins obstruction, and sinus node dysfunction. We review our 20 years of experience with Warden procedures for the repair of right partial pulmonary venous connection to the SVC., Methods: This was a single-center retrospective study of all patients (pediatric and adult) with right partial pulmonary abnormal venous connections who underwent a complete repair with the Warden procedure between 1997 and 2016. A total of 59 patients were included. The median age was 14 years (5 months to 61 years) and the median weight was 44 kg (4.9-92 kg)., Results: The mean term follow-up was 7 (±5) years. No deaths were reported. One (1.7%) patient required a pacemaker implantation; two (3.4%) suffered from a SVC stenosis successfully treated with balloon dilatation and stent implantation; and eight (13.6%) patients had transient rhythm disturbances, one had junctional ectopic tachycardia, three had sick sinus syndrome, three had an atrial flutter, and one had an atrioventricular block. None suffered from pulmonary vein obstruction., Conclusion: The Warden procedure is safe and can be performed with very low morbidity, mortality, rhythm disturbances, and decreased pulmonary and SVC obstructions., (© 2018 Wiley Periodicals, Inc.)

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 describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery., (© 2017 Wiley Periodicals, Inc.)

Background: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD)., Methods: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed., Results: The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases., Conclusion: Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery., (© 2017, Wiley Periodicals, Inc.)

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

We report a rare case of dextroversion accompanied with atrial septal defect (ASD), persistent left superior vena cava with absent right superior vena cava in a four-year-old male. A polytetrafluoroethylene (PTFE) graft as an extracardiac conduit was used to connect the persistent left superior vena cava (PLSVC) with the right atrial appendage., (© 2015 Wiley Periodicals, Inc.)

Persistent left superior vena cava (PLSVC) is the most common venous anomaly of the thorax affecting 0.5-2% of the general population. PLSVC with absent right SVC, which is also referred to as isolated PLSVC, occurs in 0.09-0.13% of patients. PLSVC should be suspected whenever a dilated coronary sinus is discovered on transthoracic echocardiography. Transthoracic echocardiography serves as an excellent modality to diagnose PLSVC. The presence of PLSVC can be confirmed by performing a bilateral "bubble study" with injection of agitated saline from both the left and the right peripheral arm veins. Although PLSVC is a benign condition, it may have important clinical implications in certain situations. We describe a case of PLSVC with an absent right SVC followed by a review of the literature and the clinical implications associated with this condition., (© 2014, Wiley Periodicals, Inc.)

Persistent left superior vena cava (LSVC) is a rare congenital anomaly which usually produces no physiologic derangements if it drains into the right atrium via the coronary sinus, but it may cause significant desaturation when it drains into the left atrium (LA). Failure to diagnose LSVC communicating with the LA preoperatively may lead to serious consequences. We are describing an interesting case of a boy who presented with systemic desaturation due to an undetected LSVC after having undergone corrective surgery for atrioventricular canal defect. We have demonstrated that echocardiography with agitated saline contrast is a simple, accurate, and inexpensive diagnostic modality., (© 2013, Wiley Periodicals, Inc.)

Residual intracardiac or extracardiac shunting is frequently seen in patients with repaired congenital heart disease and can cause systemic hypoxemia. We present the case of an adult with tetralogy of Fallot who underwent two corrective surgical procedures as a child with subsequent pulmonary valve replacement due to residual pulmonary insufficiency. Further details of her operative history were otherwise unknown. After being lost to follow-up for many years, she presented with unexplained cyanosis and a markedly abnormal agitated saline study on transthoracic echocardiography in which only the left heart filled after contrast administration. We review the differential diagnosis for such a presentation and discuss the utility of cardiac magnetic resonance imaging (MRI) to define the etiology. Cardiac MRI, a frequently used imaging modality to longitudinally assess adult patients with congenital heart disease, provides excellent image quality of cardiac structures and the ability to perform angiography in a variety of imaging planes. These advantages render cardiac MRI, a useful modality to determine the etiology of unexplained cyanosis in these patients as both intracardiac and extracardiac shunts can be detected., (© 2013, Wiley Periodicals, Inc.)

Single ventricle defects, including inflow emptying into 1 ventricle, occur at an incidence of 0.05-0.1 per 10,000 live births. The association of total anomalous pulmonary venous drainage (TAPVD) with single ventricle defects is a risk factor for poor outcome. Conversely, common atrium, a rare congenital anomaly, is an endocardial cushion defect. We present a rare case of an unoperated patient with coincidence of a single ventricle defect, TAPVD with direct drainage from the pulmonary veins into the superior vena cava, and common atrium. To the best of our knowledge, such a case has not been previously reported in the literature., (© 2013, Wiley Periodicals, Inc.)

This paper describes an uncommon case of a patient with anomaly of a persistent left superior vena cava (PLSVC). A 54-year-old man with a history of chronic kidney disease, heart failure, diabetes mellitus and hypertension was admitted to the hospital for worsening dyspnoea. During his hospital stay, heart failure was further deteriorated and he became anuric. Renal replacement therapy was then required. After multiple unsuccessful attempts of right subclavian vein catheterisation, a catheter was placed in the left subclavian vein. Chest X-ray revealed the catheter in the left side of the thorax. Transthoracic cardiac ultrasound with agitated saline and chest MRI confirmed the diagnosis of PLSVC. The patient had nine sessions of slow continuous ultrafiltration. His heart and renal function were gradually improved. Nephrologists and health care professionals must be aware of this uncommon anatomic variant. Unnecessary manipulations can lead to serious complications, such as cardiac arrhythmias, cardiac arrest and venous sinus thrombosis., (© 2012 European Dialysis and Transplant Nurses Association/European Renal Care Association.)

A 10-year-old male was admitted to our institution with complaints of mild cyanosis and dyspnea. Transthoracic echocardiography and angiography revealed a right superior vena cava (SVC) draining into the left atrium. At the time of surgery, the right SVC was connected to the right atrium., (© 2012 Wiley Periodicals, Inc.)

Anomalies of the pulmonary venous drainage vary widely in their anatomic spectrum and clinical presentation. We describe an unusual case of supra-cardiac total anomalous pulmonary venous connection (TAPVC), where the pulmonary veins drained directly in the posterior aspect of proximal right superior vena cava (SVC) through separate ostia. The veins were re-routed with a patch to the left atrium via the secundum atrial septal defect (ASD). The continuity between distal SVC and right atrium was re-established by re-implanting the SVC to the right atrial appendage (Warden Procedure)., (© 2012 Wiley Periodicals, Inc.)

A 49-year-old female who presented with 3 weeks of exertional chest pain had an abnormal mediastinal finding at chest x-ray imaging. Conventional, nongated computed tomography of the chest revealed a "mass" in proximity to the right atrium. 64-slice, cardiac gated computed tomographic coronary angiography, and transesophageal echocardiography delineated the "mass" as a coronary artery fistula structure. The fistula originated from the left main as a tubular vessel that continued into an aneurysmal sac-like cavity that emptied into the superior vena cava near the right atrium. Computed tomographic coronary angiography showed otherwise normal coronary arteries. Findings were ultimately confirmed at cardiac catheterization. Coronary steal was clinically diagnosed and she underwent surgical ligation and resection of the fistula and aneurysm. Her subsequent course was uncomplicated., (© 2011, Wiley Periodicals, Inc.)

A case of persistent left with absent right superior vena cava accompanied with atrial septal defect in a six-year-old girl is presented. This rare venous malformation was diagnosed incidentally during surgery when the pericardium was incised. An L-shape cannula was directly inserted into the left superior vena cava for cardiopulmonary bypass. The atrial septal defect was closed with a fresh autologous pericardial patch. The surgical management of this rare anomaly is the subject of this review., (© 2012 Wiley Periodicals, Inc.)

Circumflex coronary arteriovenous fistula associated with aneurysmal dilatation and draining into coronary sinus (CS) is rare. A 57-year-old female presented with progressive dyspnea and was found to have a tortuous multiloculated aneurysm of the circumflex coronary artery terminating into the CS associated with a persistent left superior vena cava. The operative repair and management of coronary arteriovenous fistula are the subject of this case report and review. , (© 2011 Wiley Periodicals, Inc.)

Background: Pulmonary hypertension and right atrial pressure overload (RAPO) cause dilation of the coronary sinus (CS). Persistent connection of the left superior vena cava (LSVC) to the CS is another cause of CS dilation. The purpose of this study was to evaluate the usefulness of coronary sinus cross-sectional area (CSA) and eccentricity index (EI) in differentiating persistent LSVC from right heart overload and RAPO in patients with dilated CS., Methods: We identified 15 patients with a dilated CS by echocardiography. Offline analysis was used to measure CS-CSA and CS-EI at end-diastole in the parasternal long axis plane. EI was defined as B/A, where A is the widest diameter and major axis of the CS, and B is the diameter of the minor axis (perpendicular to and bisecting A at its midpoint). Persistent LSVC was confirmed by either computed tomography or injection of agitated saline in the left antecubital vein., Results: CS-CSA was significantly larger in PLSVC group than in group with RAPO. Also, CS-EI was lower in PLSVC than in RAPO group (P = 0.0003). EI was the most sensitive and specific discriminator between patients with persistent LSVC vs. RAPO. CS-EI was <0.8 in all PLSVC patients and >0.8 in all RAPO patients (sensitivity and specificity = 100%)., Conclusion: Patients with persistent LSVC have a significantly higher CS-CSA than those with elevated RA pressure. When dilated CS is present, a CS-EI <0.8 is 100% sensitive and specific for persistent LSVC. Thus, the CS-EI can be used in cases of dilated CS to diagnose the presence of persistent LSVC with a very high degree of certainty, and can help differentiate this congenital anomaly from RAPO., (© 2011, Wiley Periodicals, Inc.)

Persistent left superior vena cava (PLSVC) is an infrequent abnormality. Anatomical variations can be unexpectedly identified during cardiac procedures. Modifications of surgical techniques become a must during these operations, especially during orthotopic heart transplantation. We present two cases of patients with PLSVC and discuss the anatomy, embryology, diagnosis, and modifications in transplant techniques. , (© 2011 Wiley Periodicals, Inc.)

Intravenous agitated saline injection is useful in identifying right-to-left shunting at the atrial or intrapulmonary level. Anomalous systemic venous drainage to the left atrium is a rare but easily correctable cause of right-to-left shunting which, if left undiagnosed, may have serious consequences, including meningitis and pyogenic brain abscesses. This case illustrates an unusual cause of right-to-left shunting and the utility of venous microbubble injection in its diagnosis., (© 2010, Wiley Periodicals, Inc.)

A unique arteriovenous fistula, originating from the left main coronary artery and branching to drain into the right atrium and superior vena cava is presented with review of the literature., (© 2010 Wiley Periodicals, Inc.)