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Abnormal systemic vein development produces anomalous veins, which - in the case of persistent left superior vena cava and/or left brachiocephalic vein - exhibit considerable topographic and morphometric differences in comparison with their usual anatomy. The nature and extent of those developmental anomalies - detected during intravenous procedures, such as cardiac implantable electronic device (CIED) lead insertion or central venous catheter placement - may hinder the procedure itself and/or adversely affect its outcome, both at the stage of cardiac lead advancement through an abnormally shaped vessel and lead positioning within the heart. This may lead to problems in achieving optimal sensing and pacing parameters and in ensuring that the patient cannot feel the pacing impulses. These events accompanied a de novo CIED implantation procedure in the patient with a double superior vena cava and left brachiocephalic vein agenesis, who ultimately required reoperation.

The persistent left superior vena cava (LSVC) is a common anomaly of congenital heart disease. The presence of LSVC is commonly associated with other congenital cardiac anomalies such as atrial septal defect, tetralogy of fallot, aortic coarctation, ventricular septal defect and very rarely it occurs as an isolated finding. During a routine dissection for undergraduate students, a persistent LSVC along with variation in anterior cardiac vein and right septal pouch (RSP) was observed in heart of an approximately 48-year-old male cadaver. The persistent LSVC was draining into the right atrium via coronary sinus. The persistent LSVC is usually insignificant haemodynamically as commonly it drains into right atrium via coronary sinus, but incidental finding of LSVC is important to surgeons, interventional nephrologists and radiologists before placement of central venous access device. The insertion of central venous catheter via left internal jugular vein is difficult in presence of persistent LSVC. The right superior vena cava was normal. An anterior cardiac vein joined with the right marginal vein to form a common vein. The common vein opened into the right atrium. We also observed a RSP attached to the limbus fossa ovalis inferiorly which is a kangaroo pouch–like structure. A septal pouch is potential site predispose to thrombus formation and is more common on left side. In this case report we discuss embryology, clinical significance and review of literature related to persistent LSVC, anterior cardiac vein and SP.

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.To assess the outcomes of infants with isolated persistent left superior vena cava diagnosed prenatally compared with infants with associated malformations.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.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.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.

A 35-year-old woman presented with dyspnea on exertion. The diagnosis was unroofed coronary sinus with persistent left superior vena cava and atrial septal defect. She underwent total correction with the intracardiac baffle technique by using an interatrial flap and autologous pericardial patch. Her clinical course was uneventful and markedly improved after the surgery.

Aims\ud To investigate the abnormalities of the coronary venous system in candidates for cardiac resynchronization therapy (CRT) and describe methods for circumventing the resulting difficulties.\ud \ud Methods\ud From four implanting institutes, data of all CRT implants between October 2008 and October 2020 were screened for abnormal cardiac venous anatomy, defined as an anatomical variation not conforming to the accepted ‘normal’ anatomy. Patient demographics, procedural detail, and subsequent left ventricle (LV) lead pacing indices were collected.\ud \ud Results\ud From a total of 3548 CRT implants, 15 (0.42%) patients (80% male) of 72.2 ± 10.6 years in age with an LV ejection fraction of 34 ± 10.3% were identified to have had an abnormal cardiac venous anatomy over the study period. There were 13 cases of persistent left side superior vena cava (pLSVC), five of which had coronary sinus ostium atresia (CSOA) including two with an “unroofed” coronary sinus (CS); one patient had a unique anomalous origin of the CS and one patient had an isolated CSOA. In total 14 patients (60% repeat attempt) had successful percutaneous implant under general anesthesia (46.7%) via the cephalic vein (59.1%), using the femoral approach (53.3%) for levophase venography and/or pull-through, including one case of endocardial LV implant. Pacing follow-up over 37.64 ± 37.6 months demonstrated LV lead threshold between 0.62 and 2.9 volts (pulsewidth 0.4–1.5 ms) in all cases; five patients died within 2.92 ± 1.6 years of a successful implant.\ud \ud Conclusion\ud CRT devices can be implanted percutaneously even in the presence of substantial abnormalities of coronary venous anatomy. Alternative routes of venous access may be required.

The aim of this study is to identify prenatally diagnosed cases of persistent left superior vena cava (PLSVC) in our clinic, to evaluate the associated structural and chromosomal results, and to review their outcome.During a four-year period, patients with fetal PLSVC were detected by echocardiography. We reviewed medical records of these affected pregnancies, including maternal demographics, sonographic findings, chromosomal microarray results and pregnancy outcomes.There were a total of 140 cases of fetal PLSVC. Eighty-nine fetuses (63.6%) had associated structural anomalies, while the remaining 51 fetuses (36.3%) had PLSVC as an isolated finding. In the non-isolated cases, cardiac anomalies were present in 72 fetuses (80.9%), and extracardiac abnormalities in 45 fetuses (50.6%). Among the 89 cases with non-isolated PLSVC, 12 cases had chromosomal abnormalities including 5 cases of aneuploidies. Among the 51 cases with isolated PLSVC, one pregnancy of chromosomal microduplication was detected.Isolated PLSVC is a benign vascular anomaly in low risk population. However, the information about background risk of identifying an abnormal clinically significant CMA result should be conveyed to all pregnant women when they consults this vascular variation.

While the vasodilatory and negative inotropic effects of general anesthetics are well known, their effects on the cardiac excitation and conduction systems are not fully understood. Indeed, many anesthesiologists may rarely experience cardiac arrests triggered by anesthetics without any warnings such as preceding electrocardiogram (ECG) wave form changes or extreme hemodynamic fluctuations. The authors present a case of sudden and repeated cardiac arrest during induction of general anesthesia as the first detection of sick sinus syndrome (SSS) in a patient with isolated persistent left superior vena cava (PLSVC) after a maze procedure. Careful perioperative vigilance is required for patients after the maze procedure, because general anesthetics may trigger serious bradyarrhythmias or cardiac arrest. Especially in patients with isolated PLSVC, detailed preoperative evaluation and consideration of the indications for permanent pacemaker implantation, taking into account both the risk of arrhythmias and the difficulty of emergency pacing, are recommended.

Persistent left superior vena cava, the most common thoracic venous anomaly, may complicate transvenous lead extraction (TLE). We report a successful case of TLE in a patient with persistent left superior vena cava, despite a long dwelling time and several pocket revisions due to pocket infection. The lead was removed via a hand-powered mechanical extraction sheath, and postoperative complications did not occur. Complicated TLE cases may have a better outcome if performed in a high-volume center with experienced specialists.

A 61-year-old male presented with symptoms of decompensated heart failure and cardiogenic shock. Transthoracic and transesophageal echocardiography showed severely impaired left ventricular (LV) systolic function (LVEF of 20-25%), bicuspid aortic valve with moderate aortic insufficiency and no significant stenosis, dilated coronary sinus and a tortuous vascular structure in the left-sided atrioventricular groove. Cardiac computed tomography confirmed the diagnosis of persistent left superior vena cava and a giant coronary artery fistula to the coronary sinus. Cardiac magnetic resonance illustrated non-specific late gadolinium enhancement in the mid-wall of the septum. The patient was treated medically and with cardiac re-synchronization therapy.

The superior vena cava is usually located only on the right side, but persistence of the left superior vena cavais observed in about 0.3 to 0.5 % of adults. A routine dissection of the cadaver of a 91-year-old Japanese female, whose cause of death was sepsis due to cholecystitis, was performed at Nagasaki University and revealed a double-sided superior vena cava. On the right side, the superior vena cava opened to the right atrium, while on the left, it opened into the extended coronary sinus. Veins in the left head, neck and upper limb regions joined to form the persistent left superior vena cava, with eventual drainage into the expanded coronary vein. An anastomosing branch occurred between each superior vena cava, and two thymic veins opened to the anastomosing branch. The azygos vein in the azygos venous system opened into the right superior vena cava, whereas a hemi-azygos vein opened into the azygos vein. The accessory hemi-azygos vein also opened into the azygos vein and opened cranially into the left superior vena cava. The left supreme intercostal vein also opened into the left superior vena cava. Several studies have reported a persistent left superior vena cava and the various considerations for its occurrence. Here, we propose a new hypothesis for the embryonic development of the persistent left superior vena cava with the thymic vein. This hypothesis essentially states that the left brachiocephalic vein fails to mature due to inadequate venous return from the thymic vein during the embryonic period, and the left superior vena cava then remains to maintain venous return from the left head, neck and upper limb. We also discuss the clinical significance of the persistent left superior vena cava., International Journal of Morphology, 40(1), pp.24-29, 2022

Introduction We present a case of dual drainage of the right upper lobe of the lung into the left atrium and via partial anomalous venous pulmonary return (PAPVR) into a persistent left superior vena cava (SVC). Discussion It is only in the minority of PAPVR cases where the anomalous pulmonary veins cross the midline. We provide a review of current literature on this topic and an explanatory embryological model. Knowledge of embryonic development and possible anatomic variations, including the concept of dual venous drainage of the lung, leads to better interpretation of imaging, with more accurate description of the morphology at hand. High-resolution multidetector computed tomography (MDCT) helps to delineate the exact vascular anatomy. This will enhance a better understanding of and anticipation on the patient’s disease status, with more accurate planning of intervention, and possibly less complications.

Background: Although pulmonary vein isolation (PVI) is an established procedure for atrial fibrillation (AF), non-PV foci play a crucial role in AF recurrence. Persistent left superior vena cava (PLSVC) has been reported as critical non-PV foci. However, the effectiveness of provocation of AF triggers from PLSVC remains unclear. This study was designed to validate the usefulness of provoking AF triggers from PLSVC. Methods: This multicenter retrospective study included 37 patients with AF and PLSVC. To provoke triggers, AF was cardioverted, and re-initiation of AF was monitored under high-dose isoproterenol infusion. The patients were divided into two groups: those whose PLSVC had arrhythmogenic triggers initiating AF (Group A) and those whose PLSVC did not have triggers (Group B). Group A underwent isolation of PLSVC after PVI. Group B received PVI only. Results: Group A had 14 patients, whereas Group B had 23 patients. After a 3-year follow-up, no difference in the success rate for maintaining sinus rhythm was observed between the two groups. Group A was significantly younger and had lower CHADS2-VASc scores than Group B. Conclusions: The provocation of arrhythmogenic triggers from PLSVC was effective for the ablation strategy. PLSVC electrical isolation would not be necessary if arrhythmogenic triggers are not provoked.

Background: Brain abscess is a life-threatening condition. Congenital cardiovascular malformations can create right-to-left shunting and be an underlying cause. Case presentation: A young man was admitted due to headache and deteriorating general condition. He had a history of a surgically treated brain abscess 19 years earlier. Investigations now showed a new brain abscess. The patient was operated and received a peripherally inserted central catheter in his left arm for antibiotic treatment. A chest X-ray showed abnormal positioning of the catheter lying in a persistent left superior vena cava. One day later he experienced headache and photophobia. MRI showed reoccurrence of the brain abscess and he was reoperated. Persistent left superior vena cava was considered to be the cause of the brain abscesses and he underwent endovascular embolisation and placement of a vascular plug in his left superior vena cava. Interpretation: The oxygen-rich pulmonary circulation and its immune system make it difficult for anaerobic bacteria to pass to the arterial side. In most cases persistent left superior vena cava drains into the right atrium and is asymptomatic. In 10 % of patients the persistent left superior vena cava drains directly to the left atrium and gives a right-to-left shunt. This may cause arterial bacteraemia and brain abscesses.

Background Transvenous lead extraction (TLE) is rising in parallel to cardiac implantable electronic device implantations. Persistent left side superior vena cava (PLSVC) is a relatively common anatomical variant in the healthy population; TLE in patients with a PLSVC is rare. Method Data were collated from 6 European TLE institutes of 10 patients who had undergone lead extraction with a PLSVC. Patient demographics, procedural challenges and outcomes were reported. Results Ten patients aged 73.4 ± 7.8 years (60% male) underwent TLE of 20 leads (3 left ventricle, 10 right ventricle, 7 right atrium) with dwell time of 82.95 ± 39.1 months. Of the 10 cases, 4 had an infection indication and 5 were biventricular system extractions; 25% of the extracted leads were defibrillator leads. The majority of the procedures were completed in the cardiac catheterization suite (80%) under general anaesthesia (60%) by cardiologists (80%) using a rotational powered sheath (65%). The Tandem approach was used successfully in 3 cases. Complete procedural success was obtained in 100% of cases in the absence of complications within 127.4 ± 74.7 min. There was no 30-day mortality. Conclusion TLE in PLSVC is feasible albeit rare. Standard extraction techniques in experienced hands are associated with favorable outcomes; the Tandem procedure may be an additional technique to improve the safety and efficacy of TLE in PLSVC.

Catheter ablation for atrioventricular nodal re-entrant tachycardia (AVNRT) in patients with persistent left superior vena cava (PLSVC) is challenging because of anatomical abnormalities of Koch's triangle associated with the enlarged coronary sinus ostium. We present the Case of successful ablation in a patient with PLSVC using the cryoablation technique. The ablation was successfully performed without damaging the conduction system by virtue of “cryomapping” and “cryoadhesion.” Cryoablation is a safe and efficacious alternative to radiofrequency catheter ablation for the treatment of AVNRT associated with PLSVC., Highlights • Catheter ablation for AVNRT in patients with PLSVC is challenging • We performed successful ablation in a PLSVC patient with the cryoablation technique • Ablation was successfully performed without damaging the conduction system

Background: Prenatal ultrasound (US) detection of heterotaxy syndrome can be challenging, especially in identifying cardiovascular and associated anomalies. We present a new case of heterotaxy syndrome with anomalous systemic venous return (ASVR) fully displayed at autopsy. Case report: Left heterotaxy syndrome was diagnosed in a 19 weeks' of gestation fetus with right-sided stomach. The heart showed both ventricles with left morphology, a large ventricular septal defect, persistent left superior vena cava draining into the coronary sinus, ASVR with interrupted inferior vena cava (IVC) and azygous continuation. Autopsy dissection further identified the azygous draining into the left lower pulmonary vein (LLPV). Prenatal a-CGH on villous sampling showed 22q13.1 microduplication inherited from the father, not contributory to the phenotype. Conclusion/discussion: Heterotaxy syndrome requires US accuracy for anomaly identification, as they allow legal termination of pregnancy. Our case is unusual as IVC drained into the azygous vein and then into the LLPV.

The persistence of the left superior vena cava is one of the most common abnormalities that could affect the thoracic venous return, despite its rare occurrence. It can usually be found as the only or in combination with other congenital cardiac abnormalities. Even though it is usually asymptomatic and it rarely has important consequences on the hemodynamics, it could sometimes represent a serious threat. In this regard, PLSVC often represents an incidental finding during an invasive procedure or imaging. We present an interesting case of a 66-year-old patient, with permanent atrial fibrillation and chronic kidney disease who presented to our clinic for a syncope due to complete atrioventricular block. The implant procedure was marked by the incidental intraprocedural finding of unusual venous anatomy. This anomaly included the absence of the superior vena cava, with the communication of the right brachiocephalic trunk and right subclavian vein with a persistent left superior vena cava which drainage directly into the coronary sinus. The right-side approach, as well as the limitation of using contrast-based venography, due to the kidney disease, made the procedure more difficult, but the final position of an active fixation ventricular lead was successfully achieved with optimal and stable pacing parameters through the formation of a particular curve of the lead stylet. Persistence of the left superior vena cava is a venous anomaly, which is frequently suspicioned at transthoracic echocardiography examination when the examiner found a dilated coronary sinus but diagnosed on the implant table of a cardiac device. These anomalies can pose problems and exponentially increase the procedural time even in experienced hands.

BACKGROUND Persistent left superior vena cava (PLSVC) results in a double superior vena cava (SVC), and although it is rare, this is the most common venous anomaly of the thorax. PSLVC arises from the junction of the left subclavian and internal jugular veins. It is identified on the left side of the mediastinum adjacent to the aortic arch, and it usually drains into the right atrium through the coronary sinus. This report presents the case of a 40-year-old man with an incidental finding of double SVC due to PSLVC identified on hospital admission following a motor vehicle collision. CASE REPORT A 40-year-old man was found to have a double SVC due to PLSVC upon chest radiography during hospital admission for injuries related to motor vehicle trauma. The discovery was made following placement of a central venous catheter (CVC) down the left-sided SVC and into the coronary sinus. The patient suffered no harm as a result. The diagnosis was made by chest radiography and confirmed by computed tomography angiography. CONCLUSIONS PSLVC is an uncommon condition that can complicate common procedures and therefore must be well-understood by physicians across many medical and surgical specialties. Although PLSVC may be asymptomatic, as in this case, in some patients PLSVC presents as atrial fibrillation or with nonspecific cardiac symptoms. Therefore, all patients identified with PLSVC should be investigated to exclude associated cardiac abnormalities and arrythmias, and before the placement of central venous access devices.

We herein describe a 34-year-old woman with a persistent left superior vena cava (PLSVC) impinging on the posterior wall of the left atrium, subdivided by a membrane at the distal part of the impingement. In this patient, a much dilated coronary sinus due to the PLSVC resulted in a membrane-like structure in the posterior wall of the left atrium with the appearance of thecor triatriatumin transthoracic echocardiography (ie, apseudo cor triatriatum). Although the PLSVC is a common malformation of the superior systemic vein, its aforementioned presentation is rare. Keywords:Persistent Left Superior Vena Cava,Cor Triatriatum,Coronary Sinus,Left Atrial Function

Persistent left superior vena cava (PLSVC) is a common venous variation that is usually accompanied by an absence of the left brachiocephalic vein, and displays a higher incidence in patients with congenital heart disease. Here, the case of a 57-year-old male patient who was found to have PLSVC on chest computed tomography (CT) during screening for gastric cancer metastasis at the Affiliated Hospital of Qinghai University, is described. Further coronal CT and three-dimensional reconstruction of the chest revealed the patient's double superior vena cava (DSVC), double odd veins, and left brachiocephalic vein dysplasia. The patient did not have congenital heart disease and the case was associated with dysplasia of the left brachiocephalic vein, indicating an unusual and rare venous abnormality. At the time of writing, the patient was receiving antitumour therapy.

Although rarely performed today in most centers, Senning procedure continues to be a good option for patients with transposition of great arteries presenting late with either regressed left ventricle or pulmonary hypertension. There are many subsets of patients including those having deficient atrial septal tissue, situs inversus, dextrocardia, and atrial isomerism which require complex modifications of technique. One such subset is patients having bilateral superior vena cavae (SVC), which requires coronary sinus cutback and creating a wide flap for the posterior venous baffle which unduly increases the complexity of the surgery. We describe an alternative way of rerouting a persistent left SVC by reimplanting onto the left atrial appendage which forms the part of the systemic atrium after the atrial switch surgery.

Vascular injury associated with cannulation during extracorporeal membrane oxygenation (ECMO) induction is a rare but life-threatening complication. The presence of abnormal vascular anatomy increases the risk of vascular injury and should be recognized before cannulation. We report the case of a patient with coronavirus disease (COVID-19) who was expected to undergo ECMO. By performing computed tomography (CT), we identified the absence of right superior vena cava (RSVC) with a persistent left superior vena cava (PLSVC) that could have caused serious complications associated with ECMO cannulation. PLSVC is observed in less than 0.5% of the general population; however, the combination of PLSVC and an absent RSVC in visceroatrial situs solitus is extremely rare. Attempting cannulation for Veno-venous (VV)-ECMO from the right (or left) internal jugular vein to the right atrium may cause serious complications. Cannulation may fail or lead to complications even in patients with inferior vena cava malformations. Although these vascular abnormalities are rare, it is possible to avoid iatrogenic vascular injury by identifying their presence in advance. Since anatomical variations in the vessels from the deep chest and abdominal cavity cannot be visualized using chest radiography and ultrasonography, we recommend CT, if possible, for patients with severe respiratory failure, including those with COVID-19, who may be considered for VV-ECMO induction.

In a patient requiring pacing and defibrillation therapy, but without superior venous access, combined therapy with S-ICD and leadless pacemaker could be the best solution. An appropriate programming of both devices represents the technical challenge in order to avoid inappropriate shocks due to leadless pacing oversensing.

Persistent left superior vena cava (PLSVC) poses technical challenges to implantation of transvenous cardiac implantable electronic devices. His bundle pacing is a physiologic pacing strategy to avoid or treat pacing induced cardiomyopathy. We report a case of His bundle lead implantation in a patient with PLSVC, absent right SVC, and pacing-induced cardiomyopathy. This article is protected by copyright. All rights reserved.

Background Cor triatriatum and supramitral ring are congenital anomalies which result in formation of three chambers of atria. To the best of our knowledge, simultaneous presence of both entities in the same patient resulting in the formation of four chambers of atria has not been described in the literature. Here, we report a case of simultaneous presence of cor triatriatum and supramitral ring associated with Raghib syndrome and Eisenmenger syndrome. Case presentation We report the case of a middle-aged gentleman, who presented to us with features of atrial septal defect with Eisenmenger syndrome. Multimodality imaging confirmed the simultaneous presence of supramital ring and cor triatriatum resulting in “cor tetratriatum” along with Raghib syndrome. Presence of Eisenmenger syndrome compelled us to offer medical therapy for the patient. Conclusion This is the first case report describing the simultaneous presence of supramitral ring and cor triatriatum resulting in a new entity—“cor tetratriatum”.

Background Coronary sinus (CS) ostial atresia/abnormalities prevent access to the CS from the right atrium (RA) for left ventricular (LV) lead implantation. Some patients with CS ostial abnormalities also have a small persistent left superior vena cava (sPLSVC). Objective The purpose of this study was to describe CS ostial abnormalities and sPLSVC as an opportunity for LV lead implantation and unrecognized source of stroke. Methods Twenty patients with CS ostial abnormalities and sPLSVC were identified. Clinical information, imaging methods, LV lead implantation techniques, and complications were summarized. Results Forty percent had at least 1 previously unsuccessful LV lead placement. In 70%, sPLSVC was identified by catheter manipulation and contrast injection in the left brachiocephalic vein, and in 30% by levophase CS venography. In 30%, sPLSVC was associated with drainage from the CS into the left atrium (LA). When associated with CS ostial abnormalities, the sPLSVC diameter averaged 5.6 ± 3 mm. sPLSVC was used for successful LV lead implantation in 90% of cases. In 80%, the LV lead was implanted down sPLSVC, and in 20%, sPLSVC was used to access the CS from the RA. Presumably because of unrecognized drainage from the CS to the LA, 1 patient had a stroke during implantation via sPLSVC. Conclusion When CS ostial abnormalities prevent access to the CS from the RA, sPLSVC can be used to successfully implant LV leads. In some, the CS partially drains into the LA and stroke can occur spontaneously or during lead intervention. It is important to distinguish sPLSVC associated with CS ostial abnormalities from isolated PLSVC.

Background: Persistent left superior vena cava (PLSVC) is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cortriatriatum. Objective: Aimof this case presentation was to describe a rare case of persistent left superior vena cava that is associated with atrial septal defect and partial anomalous pulmonary venous drainage. Also, how to suspect and then confirm the presence of PLSVC by echocardiographic examination, and to know the role of multi-slice CT and cardiac MRI for detection of other cardiac and extra-cardiac anomalies. Patients and methods: Male patient aged 32 year complaining of shortness of breath for about 1 year, echocardiographic examination revealed dilated coronary sinus and injection of agitated saline indicated the presence of persistent left superior vena cava. PLSVC may be associated with other anomalies so further evaluation by multi-slice CT was done to confirm the diagnosis of PLSVC as well as sinus venosus ASD and partial anomalous pulmonary venous drainage so surgical consultation was done for surgical correction. Conclusion: Diagnosis of PLSVC is important, as it is often associated with other anomalies as atrial septal defect and partial anomalous pulmonary venous drainage, which lead to pulmonary hypertension and need surgical correction before the occurrence of pulmonary hypertension.

Background Permanent cardiac implantable electronic devices (CIEDs) are traditionally implanted with the assistance of fluoroscopy. While clinically effective, this technique exposes both patients and providers to radiation which is associated with adverse health effects and represents an occupational hazard. In this study, we investigate the safety and feasibility of permanent CIED placement under the guidance of transthoracic echocardiography (TTE). There is also increasing interest in use of non‐fluoroscopic options for noninvasive cardiac electrophysiologic procedures. Methods Fifteen patients consecutively consented for initial implant of CIEDs, specifically dual chamber pacemakers (DCPM) and dual chamber implantable cardioverter defibrillators (DCICDs). Patients were excluded if they had previous implants, abandoned leads, or anatomic anomalies including congenital and known persistent left superior vena cava (PLSVC). We used TTE to guide and implant atrial and ventricular leads. Results Eleven patients received DCPMs and four patients received DCICDs. The procedure duration was 49.3 min for DCICD and 52.3 min for DCPM, p = .807. The average number of right atrial lead attempts was 1.6 for DCPMs and 1.8 for DCICD, p = .860. The average number of right ventricular lead attempts for DCPMs was 2.2 and 1.0 attempt for DCICDs, p = .044. There were no complications at 90‐day follow‐up. Conclusion We demonstrate the feasibility of TTE‐guided DCPM/DCICD implantation without use of fluoroscopy. We present this method as a safe alternative for permanent CIED placement that may reduce risk of radiation exposure and cost while maintaining safety and efficacy. No operators wore lead aprons during the procedure.

Persistent left superior vena cava (PLSVC) is the most common variant of abnormal venous return to the heart. While usually asymptomatic, it is known to complicate transvenous cardiac procedures, such as implantation of cardiac electronic devices and ablations. PLSVC can present with or without the concomitant absence of right superior vena cava (RSVC). Depending on the operator's preference, implantation of permanent cardiac pacemakers (PPMs) may be performed from the left or right side. As most often the PLSVC is only identified at the time of intervention, it follows that the variant with the absence of RSVC can be diagnosed in practice only when implanting from the right side. For this reason, the true prevalence of this variant is largely unknown because most published cases of cardiac device implantations in patients with PLSVC have been performed from the left side. We present a short 3-case series of PPM implantations in a tertiary center from the right side in patients with PLSVC and absent RSVC. We found that the use of a standard curve for ventricular lead septal placement and a wide C-curve for right atrial lead placement in these patients was a feasible technique with good outcomes.

A persistent left superior vena cava (LSVC) represents a challenging congenital abnormality for transvenous cardiac device implantation. In the current case a secondary prophylactic VDD implantable cardioverter-defibrillator (ICD) implantation was planned in a 75-year-old woman presenting with ischemic cardiomyopathy and elevated stroke risk. Since no venous communication to the right side was identified intraoperatively, the lead was placed via the persistent LSVC. The far-field signal on the floating atrial dipole could be successfully blanked out, and appropriate device function with high and stable atrial sensing was demonstrated at follow-up.Eine persistierende linke obere Hohlvene ist eine angeborene Anomalie, die anspruchsvoll hinsichtlich der transvenösen Implantation eines kardialen Geräts sein kann. Bei einer 75-jährigen Patientin mit ischämischer Kardiomyopathie und erhöhtem Schlaganfallrisiko wurde eine sekundärprophylaktische VDD-ICD-Implantation geplant (ICD implantierbarer Kardioverter-Defibrillator). Da intraoperativ keine venöse Verbindung zur rechten Seite festzustellen war, wurde die Elektrode über die persistierende linke obere Hohlvene gelegt. Das Fernfeldsignal am flottierenden atrialen Dipol konnte erfolgreich ausgeblendet und eine adäquate Gerätefunktion mit guter und stabiler atrialer Wahrnehmung in der Nachsorge nachgewiesen werden.

Upper venous system anatomic variations may cause difficulties during cardiac pacemaker implantation. Persistent left superior vena cava (PLSVC) and absent right superior vena cava could be an arrhythmogenic source of atrial arrhythmias and cardiac conduction disease. We represent dual-chamber pacemaker implantation in a patient with a very rare upper venous system anomaly, paroxysmal atrial fibrillation, sick sinus syndrome, that cause unusual fluoroscopic image.

Persistent left superior cava mostly drains into the right atrium via the coronary sinus. It rarely drains into the left atrium. Extracardiac and intracardiac repair techniques have been described for the repair of persistent left superior vena cava draining into the left atrium. Herein, we report the successful application of a new intracardiac repair technique by using a turned-in left atrial appendage in a 3-year-old male patient with a persistent left superior vena cava draining into the left atrium.

BACKGROUND: Structural features of the patients vascular system can cause unintended complications when providing vascular access and can disorient the specialist in assessing the location of the installed catheter. This study aimed to demonstrate anatomical features of the vascular system of the superior vena cava and diagnostic steps when providing vascular access in a child. CASE REPORT: Patient K (3 years old) was on planned maintenance of long-term venous access. Preliminary ultrasound examination of the superior vena cava did not reveal any abnormalities. Function of the right internal jugular vein under ultrasound control was performed without technical difficulties; a J-formed guidewire was inserted into the vessel lumen. X-ray control revealed its projection in the left heart, which was regarded as a technical complication, so the conductor was removed. A further attempt to insert a catheter through the right subclavian vein led to the same result. For a more accurate diagnosis, the child underwent computed angiography of the superior vena cava system. Congenital anomalies of the vascular system included aplasia of the superior vena cava and persistent left superior vena cava. Considering the information obtained, the Broviac catheter was implanted under ultrasound control through the left internal jugular vein without technical difficulties with the installation of the distal end of the catheter into the left brachiocephalic vein under X-ray control. CONCLUSION: A thorough multifaceted study of the vascular anatomy helps solve the anatomical issues by ensuring vascular access and preventing the risks of complications.

Catheter ablation of cardiac arrhythmias is usually performed through the femoral venous approach. Systemic venous return anomalies such as interruption of the inferior vena cava may represent a challenge during electrophysiological procedures. A 55-year-old patient with previous surgical correction of abnormal pulmonary venous return was admitted for poorly tolerated atrial flutter recurrences. He also had an interrupted inferior vena cava continuing as azygos vein and left superior vena cava draining via coronary sinus into the right atrium. Cavotricuspid isthmus radiofrequency ablation was successfully performed through the persistent left superior vena cava using a three-dimensional (3D) electroanatomical mapping system. Despite systemic venous abnormalities may potentially have important implications during electrophysiological procedures, arrhythmias can be successfully ablated with the aid of 3D electroanatomical mapping systems.

Background: Presented is a case of persistent left superior vena cava draining into the right atrium through coronary sinus and finally opens into right atrium. Abnormalities of the vascular system are more commonly seen due to its importance in circulation. Persistent left superior vena cava is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress.The venous anomaly of a persistent left superior vena cava (PLSVC) affects 0.3%–0.5% of the general population. Normally the superior vena cava is a single vascular structure formed by the union of right and left brachiocephalic veins which are in turn formed by the union of internal jugular and subclavian veins of corresponding side, draining the head and neck as well as the superior extremity. Aims and Objective: To evaluate the accuracy of persistent left superior vena cava and to find out the opening of PLSVC and formations of both SVC. Materials and Methods: During routine dissection of Thorax, we have opened the thoracic cage and take out the Heart. during that we found separate SVC and then we did the study on this PLSVC in the Department of Anatomy, Jaipur National University Institute for Medical Sciences and Research Centre (JNUIMSRC) Jaipur and National Institute of Medical Sciences and Research (NIMS & R). Results: We found persistent left superior vena cava in two cadavers out of 30 cadavers (6.66%) one was 64-year-old male cadaver and another 72-year-old male cadaver. Both the vena cavae were formed as of brachiocephalic veins of the corresponding side. The persistent left superior vena cava opened into the enlarged coronary sinus that drained into the right atrium between the opening of inferior vena cava and right atrio-ventricular orifice. Conclusion: It has important clinical implications in certain clinical interventions. It may complicate placementof cardiac catheters or pacemaker leads.

RETRACTEDTA clinical case of successful orthotopic heart transplantation using the biatrial technique with the displacement of the persistent left superior vena cava into the right atrium. The achieved clinical effect as a result of the treatment fully justifies the chosen surgical tactics and allows us to recommend the proposed tactics for the treatment of such a rare pathology.

Objectives The aim of this study is to share our experience in the prenatal diagnosis and characteristics of double aortic arc and neonatal consequences. Methods We retrospectively analyzed 2153 fetal echocardiography reports between 2014 and 2019 years. Records of 14 fetuses with double aortic arc were examined. Prenatal and postnatal medical records, sonographic images, genetic reports, associated cardiac and extracardiac anomalies, and neonatal clinical results of affected fetuses were reviewed retrospectively. Results DAA was isolated in 9 of 14 (64.2%) cases, while the other five cases had cardiac or extracardiac accompanying ultrasound findings. Three of cases (21.4%) were associated with other heart pathologies, including ventricular septal defect, double outlet right ventricle, and persistent left superior vena cava. In 10 cases (71.43%), the right aortic arch diameter was dominant. The left aortic arc was dominant in two cases and both arcs were symmetrical in the remaining two cases. 22q11 microdeletion was the only chromosomal abnormality and was detected in two of nine patients who accepted genetic analysis. Intrauterine fetal death occurred in two of the cases. After birth, in 58.3% (7/12) of the live born cases various degrees of symptoms. Surgical repair was performed with the division of the aorta to all symptomatic cases at different times according to severity of the complaints. Conclusions Since it can cause severe respiratory distress in the postnatal period and may accompany chromosomal anomalies, it is important to provide antenatal diagnosis of double aortic arc and adequate counseling to the family.

Objective We reported a fetus that presenting with persistent left superior vena cava (PLSVC), polyhydramnios, and a small gastric bubble during prenatal examination and identified VACTERL association after birth. Case report A 34-year-old woman underwent amniocentesis at 18 weeks of gestation because of advanced maternal age and the result was normal. Subsequently, an ultrasound revealed single umbilical artery (SUA) at 21 weeks of gestation. She received a detailed fetal anatomy survey that presented the same findings and PLSVC. A small visible gastric bubble was noted at that time, and the other organs were unremarkable. Polyhydramnios was identified at 30 weeks of gestation and amnioreduction was subsequently performed at 32 weeks of gestation. However, polyhydramnios was persisted despite amnioreduction and intrauterine growth restriction was also detected. A cesarean section was performed because of fetal distress at 36 + 2 weeks, and a 1832-g female baby was delivered. Pre-axial polydactyly at left thumb, SUA and esophageal atresia with distal tracheoesophageal fistula (TEF) were identified after birth. The neonate died at age of 4 days because of surgical complication following esophageal anastomosis. Conclusion Prenatal diagnosis of PLSVC associated with polyhydramnios and a small gastric bubble may indicate esophageal atresia with TEF, and further examination for associated syndromes such as VACTERL association is warranted.

A persistent LSVC with an absent right superior vena cava (RSVC) is unusual, encountered in about 10% of patients with persistent LSVC. We present 14 patients with a prenatal diagnosis of single LSVC and situs solitus, including prenatal findings and information on medium-term follow-up. We identified patients with situs solitus and a persistent LSVC born between March 2004 and March 2020, which had been diagnosed prenatally between December 2003 and November 2019. From this cohort, we identified those with absent RSVC. In the population of women undergoing fetal echocardiography, the prevalence of persistent LSVC in situs solitus was 0.43% (84/19,712). For the 84 identified patients and for the entire population respectively, 14/84 (17%) and 14/19,712 (0.07%) had a single LSVC (absent RSVC). Of 14 patients with a single LSVC, 8 (57%) were male. For the 84 identified patients and for the entire population respectively, 70/84 (83%) and 70/19,712 (0.36%) had bilateral SVCs. For a single LSVC (absent RSVC) and situs solitus, the majority had no associated cardiac, extracardiac, or syndromic abnormalities.

Objective: to present our own experience of heart transplantation in a patient with persistent left superior vena cava (PLSVC). A clinical case of successful orthotopic heart transplantation using the biatrial technique in a patient with PLSVC drainage into the right atrium is presented. The clinical effect achieved as a result of the treatment fully justifies the chosen surgical tactics and allows us to recommend the proposed tactics for treatment of such a rare anomaly. Conclusion. The clinical effect achieved as a result of the treatment fully justifies the chosen surgical tactics and allows us to recommend the proposed tactics for treatment of such a rare anomaly.

Persistent left superior vena cava (PLSVC) is the most common venous anomaly with an incidence of 0.3–0.5% in the general population. Here, we report a rare case of PLSVC with anomalous atrium in a cadaver during the student′s dissection session at the University of Tsukuba. In this case, the coronary sinus had merged with the right atrium to form an enlarged sac-like structure and received systemic venous flow including inflow from the PLSVC. The roof of the coronary sinus with the right atrium was thicker than that of the control cases. We further found that the distance between the sinoatrial node and the opening of the coronary sinus was slightly more than half of that in control cases. This variant appears interesting and is worth reporting for developmental and clinical consideration., Published online 24 January 2021.

Requiring bilateral superior cavopulmonary anastomosis (bSCPA) instead of unilateral superior cavopulmonary anastomosis (uSCPA) could influence surgical timing and outcomes. We compared surgical timing and outcomes for patients who underwent uSCPA to those who underwent bSCPA through use of the Pediatric Heart Network’s public datasets for the Infant Single Ventricle trial and Single Ventricle Reconstruction trial. There was no statistically significant difference in median age at SCPA (158 vs. 150 days, p = 0.68), hospital length of stay (LOS) (7 vs. 7 days, p = 0.74), intensive care unit (ICU) LOS (4 vs. 5 days, p = 0.53), time requiring ventilator support (2 vs. 2 days, p = 0.51), or oxygen saturation at discharge (82 vs. 81%, p = 0.22) between the uSCPA and bSCPA groups, respectively. However, sub-analysis comparing only those who underwent early SCPA, at

A persistent left superior vena cava (PLSVC) is a rare malformation which affects approximately 0.3% - 0.5% of the population and it is presented along with a right-sided superior vena cava in 82.2% of the cases reported [1]. Clinicians diagnose it incidentally by difficulties with pacemaker implantation, central venous catheterization or screening for another etiologies when it is not accompanied by other anomalies it is typically asymptomatic. W. Schummer et al. described the embryogenesis and the anatomic variations of persistent LSVC according to the positioning of a central venous catheter on the chest radiograph: type I, normal; type II, only PLSVC; type IIIa, right and left superior vena cava with connection; type IIIb, right and left superior vena cava without connection [2]. In 92% of individuals with PLSVC, the PLSVC drains into a dilated coronary sinus (CS) and rest 8% drain directly into the left atrium. PLSVC is caused by a failure in the closure of the left anterior cardinal vein during embryogenic development [3]. The coronary sinus (CS) is a vein that transmits venous blood to the right atrium though atrioventricular groove. The CS wall contains atrial myocardium. Thus, its size extensively depends on variability of blood flow and pressure. We present a variant PLSVC with unknown prevalence and a mild platypnea-orthodeoxia syndrome after recovery of COVID-19 related acute respiratory distress syndrome (ARDS).