Challenge Accepted: Lead Extraction in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Occlusion.

Persistent left superior vena cava (PLSVC) is an anatomic variant that is relatively uncommon in the general population. Lead extraction through PLSVC is extremely rare. Due to unusual anatomy, the procedure carries challenges that require special considerations and careful planning. The authors report a case of lead extraction through a PLSVC with occluded right superior vena cava and highlight the challenges and outcomes of the procedure.

Absent right superior vena cava with persistent left superior vena cava in a patient with COVID-19.

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.

Multiple thromboembolic events associated with bilateral superior vena cava and anomalous drainage into the left atrium.

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.

Rare case of right ventricular dilatation associated with anomalous pulmonary venous drainage, sinus venosus atrial septal defect and persistent left superior vena cava.

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.

Modified bi-caval view-An ideal view for diagnosing left superior vena cava with large left innominate vein on saline contrast echocardiography.

Left superior vena cava (LSVC) is the most common congenital thoracic venous anomaly which commonly drains into the right atrium via the coronary sinus. Various clinical implications are associated with LSVC and is commonly diagnosed with saline contrast echocardiography. In this case we discuss the importance of a modified bi-caval view over the mid-oesophageal four-chamber view in diagnosing LSVC with the large left innominate vein.

Left upper lung cancer with persistent left superior vena cava and left azygos vein: a case report.

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.

Persistent left superior vena cava with absent right superior vena cava in adults: CT and clinical findings.

PURPOSE: Persistent left superior vena cava (PLSVC) with absent right superior vena cava (ARSVC) is rare. We evaluated CT and clinical findings of adults with ARSVC. MATERIALS AND METHODS: Our study included 11 adults with ARSVC (mean age, 63 ± 17 years). We evaluated CT findings of the azygos vein system and coronary sinus (CS) using thin slice data of > 64 MDCT. Arrhythmia and congenial heart disease were assessed. We compared the CS diameters between the ARSVC group and the control group of 120 adults with normal vena cava (mean age, 60 ± 4 years). RESULTS: Adults with ARSVC had no azygos vein arch and the hemiazygos vein continued to PLSVCs via the superior intercostal vein. Eight adults had arrhythmia including conduction disturbance and one arrhythmia was associated with atrial septal defect. All adults with ARSVC had dilated CS larger than 11 mm. The mean diameter of the CS in the ARSVC group was 18.4 mm, and that of the control group was 6.8 mm. CS diameter in the ARSVC group was larger than that in the control group (p < 0.001). CONCLUSION: Adults with ARSVC had dilated CS and no azygos vein arch. Four adults had conduction disturbance.

Persistence of left superior vena cava detected during cuffed dialysis catheter insertion.

Persistent left superior vena cava is a rare finding seen only in 0.3%-0.5% population. It is generally asymptomatic and is often discovered after central venous catheterization done for various indications. We present a case where we demonstrate persistent left superior vena cava/left cardinal vein remnant discovered during left-sided cuffed catheter insertion for hemodialysis in a 65-year-old chronic kidney disease patient. Findings were confirmed with computed tomography venogram. This anomaly poses iatrogenic risks to the patient if not detected early. A catheter along the left mediastinal border can be dangerously close to descending aorta and could also be indicative of its placement in the internal thoracic vein or, pericardiophrenic vein. It can also perforate the persistent/remnant vessel and enter the pleura, pericardium, or mediastinum. This case emphasizes the importance of familiarity with this entity and its role in determining the appropriate venous access for patient therapy when faced with this clinical situation.

Persistent Left Superior Vena Cava in a Heart Transplant Recipient's Native Heart: A Rare Case Diagnosed by Transesophageal Echocardiogram.

Persistent left superior vena cava (PLSVC) is a rare abnormality with incidence reported as 0.3% to 0.5% in the general population and about 10 times higher in patients with congenital heart disease. The diagnosis of PLSVC in native or donor hearts of patients undergoing heart transplants has been reported in surgical journals. However, this rare finding has not been described in similar heart transplant settings in anesthesia literature. This case describes a 44-year-old male orthotopic heart transplant recipient who was incidentally diagnosed with PLSVC in his native heart on transesophageal echocardiogram after a central venous catheter placement. The particular position of the central venous catheter, in our case, raised the suspicion of PLSVC but needed further verification. With the help of images and videos, we demonstrate that transesophageal echocardiogram can be instrumental in diagnosing PLSVC. Furthermore, the case highlights the importance of effectively communicating with the surgeon about such a finding so that the surgical plan can be modified in a timely manner.

Double Anomalies: Brugada Syndrome Presenting with a Persistent Left Superior Vena Cava.

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.

Cardiac implantable electronic devices in patients with persistent left superior vena cava-A single center experience.

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.

Impact of persistent left superior vena cava on radiofrequency catheter ablation in patients with atrial fibrillation.

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.