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We present a case report detailing the surgical intervention in a patient with prosthetic aortic valve endocarditis complicated by a paravalvular abscess extending to the mitral-aortic fibrosa. Urgent surgery was required due to severe detachment of the prosthetic aortic valve, marking her third cardiac surgical procedure. Notably, preoperative imaging revealed the presence of a persistent left superior vena cava, a rare vascular anomaly requiring specialized cannulation techniques. The surgical approach involved removal of the infected tissue and prosthetic valve, followed by replacement with a cryopreserved aortic homograft, chosen for its anatomical adaptability., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

A 51-year-old woman presented with recurring palpitations. Electrocardiography revealed narrow QRS tachycardia with short RP configuration. Computed tomography showed coronary sinus (CS) ostial atresia along with a small persistent left superior vena cava (PLSVC). Electrophysiological study identified the retrograde earliest atrial activation site (EAAS) at the CS ostium without decremental properties, and para-Hisian pacing suggested retrograde atrioventricular nodal conduction. Using a 1.6-Fr microelectrode catheter distally placed in the CS via the PLSVC, EAAS was confirmed within the left atrium, not the CS ostium. Transseptal approach revealed a left lateral accessory pathway, which was successfully eliminated., (© 2024 Wiley Periodicals LLC.)

Intrahepatic interruption of the inferior vena cava (IVC) with continued hemizygous is a very rare abnormality and sometimes it may be accompanied by other cardiovascular abnormalities. Continuation of the hemizygous vein draining into the right atrium through the left superior vena cava (LSVC) is much rarer. In this paper, we have presented a patient who had simultaneous IVC interrupted with persistent LSVC and suffered from Atrioventricular nodal reentrant tachycardia (AVNRT). Finally, radiofrequencies (RF) catheter ablation for AVNRT was successfully performed through a left subclavian vein access., (© 2024. The Author(s).)

Abstract: Isolated persistent left superior vena cava (PLSVC) is a very rare congenital thoracic venous system anomaly and is commonly an incidental finding, usually detected during central venous access, cardiac catheterization, or cardiothoracic surgeries. This is a rare case report wherein the patient is a known case of ischemic heart disease with s/p percutaneous transluminal coronary angioplasty (PTCA) with a stent to left anterior descending (LAD) artery with in-stent re-stenosis presented with complete heart block and had an unanticipated discovery of isolated PLSVC on facing difficulty during the transvenous approach of permanent pacemaker implantation (PPI). In this case report, we inspect the challenges associated with and various clinical implications of isolated PLSVC., (Copyright © 2024 Copyright: © 2024 Annals of Cardiac Anaesthesia.)

Guidewire loss is a rare complication of central venous catheterization. A 65-year-old male was hospitalized in a high-dependency unit for exacerbation of chronic obstructive pulmonary disease, pneumonia, erythrocytosis, and clinical signs of heart failure. Upon admission, after an unsuccessful right jugular approach, a left jugular central venous catheter was placed. The next day, chest radiography revealed the catheter located in the left parasternal region, with suspected retention of the guidewire, visually confirmed by the presence of its proximal end inside the catheter. The left parasternal location of the catheter and the typical projection of the guidewire in the coronary sinus, later confirmed by echocardiography, raised suspicion of a persistent left superior vena cava (PLSVC). Agitated saline injected into the left antecubital vein confirmed bubble entry from the coronary sinus into the right atrium. After clamping the guidewire, the catheter was carefully retrieved along with the guidewire without any complications. This is the first reported case of guidewire retention in PLSVC and coronary sinus. It underscores the potential causes of guidewire loss and advocates preventive measures to avoid this potentially fatal complication., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© 2024 Palić et al.)

Background: Isolated agenesis of ductus venosus (ADV) is usually a benign condition, but it may be associated with cardiovascular defects, hydrops, growth restriction, and chromosomal abnormalities. Additionally, persistent left superior vena cava (PLSVC) and bovine aortic arch are relatively common fetal anomalies. To the author's knowledge, this is the first report of prenatal detection of DV agenesis and PLSVC associated with the postnatal bovine aortic arch with a hypoplastic transverse aortic arch., Case: A 25-year-old, G2P1 woman was referred to our department at 31 weeks due to fetal growth restriction and short femur. On fetal echocardiography, DV could not be viewed via two-dimensional (2D) and Doppler ultrasound (US) imaging; there was also evidence of the co-occurrence of PLSVC and an aortic arch anomaly. We revealed the intrahepatic continuation of the umbilical vein. A weekly follow-up program was scheduled for the patient and the rest of the pregnancy was uneventful. Postnatal, thorax computer tomography and transthoracic echocardiography (TTE) demonstrated PLSVC and bovine aortic arch associated with hypoplastic transverse aortic arch. Routine echocardiographic examinations revealed that the blood flow of the aortic arch had increased gradually, and the male infant's aortic arch had significantly widened and reached the normal range until the baby was discharged from the hospital., Conclusion: DV agenesis and PLSVC are usually benign conditions but underlying serious heart diseases may accompany them. Therefore, in situations like ours, a prenatal aortic arch evaluation is of capital importance. Postnatal hemodynamic changes should be taken into consideration in the management of these cases. This is the first example in the literature that these abnormalities co-existed in one case., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

A persistent left superior vena cava (PLSVC) is a commonly observed anatomical anomaly that is frequently detected incidentally via computed tomography (CT) imaging. However, the occurrence of a PLSVC with a right superior vena cava (RSVC) defect, also known as "isolated PLSVC," is a much rarer anomaly. This peculiar malformation can lead to sinoatrial dysfunction, thus necessitating pacemaker implantation, which requires delicate manipulation due to various anatomical complexities. We herein present a case of a sick sinus syndrome with this rare anomaly, which required special consideration when performing pacemaker lead placement.

Competing Interests: Disclosures None.

Competing Interests: Declaration of Competing Interest The authors have no competing interests to declare.

Background: Persistent left superior vena cava (PLSVC) is the most prevalent form of thoracic venous abnormality and can serve as a significant arrhythmogenic source in atrial fibrillation (AF)., Methods and Results: Among the 3950 patients who underwent radiofrequency ablation for AF between September 2014 to April 2020, 17 patients (mean age 59.4 ± 8.0 years, 64.7% male) with PLSVC were identified. Among them, nine patients (52.9%) had a prior history of pulmonary vein isolation (PVI) alone. Eight out of nine patients who experienced AF recurrence underwent PLSVC isolation with or without pulmonary vein (PV) reconnection. For the remaining eight patients (47.1%), PVI plus PLSVC isolation were performed during the index procedure. Ectopy originating from PLSVC was documented in 11 patients (64.7%) and successful PLSVC isolation was achieved in 16 patients (94.1%). After a median follow-up of 28.3 months, freedom from AF/ atrial tachycardia (AT) was observed in 13 patients (76.5%)., Conclusion: Empirical PLSVC isolation beyond PVI appears to be a feasible and safe strategy to prevent AF recurrence in patients with concomitant PLSVC., (© 2023 Wiley Periodicals LLC.)

Competing Interests: Disclosures None.

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

Background: Wolff-Parkinson-White syndrome is characterized by a short PR interval (delta-wave), long QRS complex, and the appearance of paroxysmal supraventricular tachycardia. Patients with Wolff-Parkinson-White syndrome usually have one accessory pathway, whereas cases with multiple accessory pathways are rare. Persistent left superior vena cava is a vascular anomaly in which the vein drains into the right atrium through the coronary sinus at the junction of the left internal jugular and subclavian veins due to abnormal development of the left cardinal vein. The simultaneous presence of multiple accessory pathways and persistent left superior vena cava has not been reported before., Case Presentation: A 56-year-old Japanese man with a 5-year history of palpitations was referred for radiofrequency catheter ablation due to increased frequency of tachycardia episodes in the previous 2 months. Persistent left superior vena cava was confirmed by transthoracic echocardiography and computed tomography. An electrophysiological study revealed that the accessory pathways were located in the left lateral wall, anterolateral wall, and posteroseptal region. They were completely ablated with radiofrequency energy application., Conclusions: We reported an extremely rare case of a patient with multiple accessory pathways and persistent left superior vena cava. Our case may suggest a potential embryological relationship between the multiple accessory pathways and persistent left superior vena cava., (© 2023. The Author(s).)

BACKGROUND Castleman disease was first described in 1956 as mediastinal masses composed of benign lymphoid hyperplasia with germinal center formation and capillary proliferation closely resembling thymomas. It has been linked with many multi-system disorders, including myasthenia gravis. Cases of Castleman disease with corresponding myasthenia gravis have higher rates of postoperative myasthenic crisis, which are reported as high as 37.5%. We encountered a case of Castleman disease with myasthenia gravis that was discovered early and managed successfully with complete surgical resection and no postoperative myasthenic crisis. CASE REPORT A 25-year-old woman with an uncomplicated history presented with shortness of breath, numbness in hands, tiring with chewing, and fatigue. Myasthenia gravis was diagnosed with serology test results, and a 7.5×7.0-cm mediastinal mass was discovered in addition to the incidental finding of a persistent left superior vena cava, closely abutting the mass. Biopsy showed lymphoid proliferation, regressed germinal centers surrounded by small lymphocytes, and vascular proliferation, consistent with unicentric Castleman disease, hyaline-vascular type. The patient was successfully treated for Castleman disease with myasthenia gravis, and no postoperative myasthenic crisis occurred. CONCLUSIONS Castleman disease associated with myasthenia gravis can dramatically increase the risk of postoperative myasthenic crisis. Our literature review of all 16 cases of Castleman disease with myasthenia gravis since 1973 revealed that 18.75% of cases were associated with a postoperative myasthenic crisis. This association elicits the importance of prompt diagnosis of myasthenia gravis when evaluating mediastinal masses and the value of having neurology and anesthesiology staff aware of the increased risk of crisis.

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., (© 2021 Wiley Periodicals LLC.)

Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath in the upright position that is relieved with supine positioning. We present a rare case of persistent left superior vena cava (PLSVC) draining into the left atrium causing a right-to-left shunt and subsequent POS. A 30-year-old female with a past medical history of hypertrophic cardiomyopathy, congenital Long QT syndrome and a left-sided dual chamber implantable cardioverter-defibrillator (ICD) presented with dyspnea and lightheadedness. Prior to presentation, the patient underwent a left-sided ICD extraction due to ICD lead infection and re-implantation from the right side through the cephalic vein. After further investigation, it was concluded that the PLSVC resulted in a physiological right-to-left shunting causing POS, with resolution of her symptoms after surgical ligation. To our knowledge, this is the first case report of PLSVC presenting with POS without anatomical intracardiac shunts following iatrogenic right superior vena cava (RSVC) obstruction., (© 2021 Wiley Periodicals LLC.)

A 49-year-old female patient presented with acute-on-chronic chest pain. She was diagnosed with multiple systemic thromboemboli, including myocardial infarctions, bilateral chronic pulmonary emboli, ischaemic stroke, deep venous thrombosis and superficial thrombophlebitis. She had a background of sickle cell trait. Cardiac magnetic resonance showed bilateral superior vena cava (SVC). The right-sided SVC (RSVC) was joined by the right upper pulmonary vein and drained anomalously into the left atrium. This caused a small volume right to left shunt. The persistent left SVC drained into the right atrium (RA) via a dilated coronary sinus. The overall clinical impression was recurrent paradoxical emboli due to anomalous venous anatomy with a thrombophilia secondary to sickle cell trait. In the normal embryo, the right common cardinal vein develops to become the RSVC, which drains into the RA by term., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 34-year-old woman was seen in the emergency department for shortness of breath and chest pain. During a pandemic, it is easy to 'think horses and not zebras', and with a patient presenting with the classic coronavirus symptoms it would have been easy to jump to that as her diagnosis. After a careful history and examination, it became clear that there was another underlying diagnosis. Chest X-ray, echocardiogram and CT scan revealed marked right ventricular dilatation and pulmonary hypertension, alongside a persistent left superior vena cava (PLSVC). Further investigation with cardiac MRI and coronary angiography at a tertiary centre demonstrated that she not only have a PLSVC but also a partial anomalous pulmonary venous drainage and sinus venosus atrial septal defect. This case highlights the importance of considering all differentials and approaching investigations in a logical manner., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Persistent left superior vena cava (PLSVC) is the most common congenital intrathoracic venous anomaly with significant clinical relevance. In the vast majority of cases, it is asymptomatic and diagnosed after noticing an abnormal course of central venous access device on a routine post-procedure roentgenogram. It may also be accidentally discovered after facing difficulty in accessing the right side of the heart from a left internal jugular vein or left subclavian vein approach, a common site of access while placing cardiac pacemaker and Swan-Ganz catheter, or after a complication associated with hemodialysis (HD) catheter insertion. HD through a catheter in PLSVC has its own set of pitfalls and should be reserved for short-term dialysis at the best. In this case report, we present a scenario where PLSVC was discovered after the placement of a tunneled HD catheter.

Background: With the popularization of thoracoscopic surgery, more and more macrovascular malformations have been reported. Understanding some vascular malformations with relatively fixed anatomical site and their range of drainage could avoid severe complications during the surgery. Persistent left superior vena cava (PLSVC) is a common thoracic vascular malformation, and is always combined with other cardiovascular dysplasia. As for the patient with upper left lung cancer in this case, he had PLSVC and left azygos vein, and non-metastatic enlargement of the lymph nodes at the same time, which had influenced the decisions on surgery and treatment. We made a summary of experience regarding this., Case Presentation: A 46-years-old male patient, his CT found a space-occupying lesion in the superior lobe of the left lung. The chest CT showed that the patient had PLSVC and left azygos vein, and multiple enlarged lymph nodes in the mediastinum. The patient received thoracoscopic upper left lung lobectomy and lymph node dissection. It was discovered that the left azygos vein had a concealed form, which influenced the lymph node dissection. The post-surgery pathology showed that there was squamous cell carcinoma in the upper left lung (pT2bN0M0 p Phase IIA) and no cancer metastasis with the lymph nodes. The patient had a good post-surgery recovery., Conclusions: PLSVC is not rare, and is always combined with other vascular malformations. If discovering PLSVC before surgery, we suggest completing chest enhanced CT and vascular reconstruction, to find out other cardiovascular malformations that may exist. Left azygos vein is a rare vascular malformation, but it has a relatively fixed anatomical site, and always co-exists with PLSVC, therefore, understanding anatomy of left azygos vein is good for preventing accidental damage. Especially when performing surgery above the left pulmonary artery trunk, attention shall be paid to preventing damage to the left azygos vein. In addition, as for the patient with the diagnosis of lung cancer before surgery, it is not reliable to judge whether there is metastasis or not merely according to the size of the lymph nodes, instead, PET-CT or needle biopsy is recommended.

BACKGROUND The presentation of Brugada syndrome (BrS) with a persistent left superior vena cava (PLSVC) is expected to be a rare entity. It is unknown if this venous anomaly is linked to the arrhythmogenesis seen in BrS, or it is coincidental. This case describes a clinical presentation of the 2, in tandem, and displays the anomaly in association with BrS. CASE REPORT A 54-year-old female presented to the Emergency Department with non-prodromal syncope. This was on a background of a number of similar episodes in the past, and a current suspected viral illness comprising fever and diarrhea. Her resting electrocardiogram was suggestive of BrS. The later was confirmed with an ajmaline provocation test after ECG normalization in the subsequent 24 hours post admission. Pre-intracardiac defibrillator (ICD) procedure imaging displayed the PLSVC. An ICD was implanted, and the advancement of the guidewires displayed the venous anomaly. Post-procedure echocardiography confirmed appropriate positioning of the leads. The patient recovered well and is currently symptom free. CONCLUSIONS PLSVC presenting with BrS is a rare occurrence. It is unknown whether or not the PLSVC and BrS are linked in their presentation, or merely a coincidence.

Background: There are limited data on cardiac implantable electronic device implantation (CIED) in patients with persistent left superior vena cava (PLSVC)., Objective: To describe the outcomes of implanting CIEDs with a focus on cardiac resynchronization therapy (CRT) in patients with PLSVC., Methods: We identified all patients with a PLSVC that underwent CIED implantation from December 2008 until February 2019 at our institution by querying the electronic medical record (n = 34). We then identified controls in a 3:1 fashion (n = 102) by matching on device type (CRT vs non-CRT). Procedure success, complications, fluoroscopy and procedural time were recorded. Outcomes were compared using a two-way analysis of variance test and conditional regression modeling for continuous and categorical variables, respectively., Results: A total of 34 patients with PLSVC underwent 38 procedures. Four patients underwent dual chamber system implantation followed by a subsequent upgrade to CRT. Thirteen patients underwent CRT implantation: one was implanted via the right subclavian while the rest were implanted via the PLSVC. Left ventricular (P = .06). Procedure and fluoroscopy times were significantly higher in the PLSVC as compared with the control group (97.7 vs 66.1 minute, P < .001 and 18.1 minute vs 8.7 minutes, P = .005, respectively)., Conclusion: CIED implant in patients with PLSVC is feasible but technically more challenging and appears to be associated with higher risk of right ventricular lead dislodgment., (© 2020 Wiley Periodicals, Inc.)

Aims: The impact of persistent left superior vena cava (PLSVC) in atrial fibrillation (AF) patients undergoing radiofrequency catheter ablation (RFCA) is not well known. We performed this analysis to evaluate the electrophysiological characteristics of PLSVC and its role in triggering and maintaining AF., Methods and Results: Patients with AF referred to two tertiary hospitals were screened and patients with PLSVC in pre-RFCA imaging studies were enrolled. Among 3967 patients, PLSVC was present in 36 patients (0.9%). There were four morphological types of PLSVC: type 1, atresia of the right superior vena cava (SVC) (n = 2); type 2A, dual SVCs with an anastomosis between right and left SVCs (n = 15); type 2B, dual SVCs without an anastomosis (n = 16); type 3, PLSVC draining into the left atrium (LA; n = 2); and unclassified in one patient. Thirty-two patients underwent RFCA and electrophysiology study focusing on PLSVC: PLSVC was the trigger of AF in 48.4% of patients and the driver of AF in 46.9% of patients. Cumulatively, PLSVC was a trigger or driver of AF in 22 patients (68.8%). Whether to ablate PLSVC was determined by the results of electrophysiology study, and no significant difference in the late recurrence rate was observed between patients who did and did not have either trigger or driver from PLSVC., Conclusion: Pre-RFCA cardiac imaging revealed PLSVC in 0.9% of AF patients. This study demonstrated that PLSVC has an important role in initiating and maintaining AF in substantial proportion of patients. Electrophysiology study focusing on PLSVC can help to decide whether to ablate PLSVC., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.)