Persistent left superior vena cava isolation in patients with atrial fibrillation: Selective or empirical?

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.

Multiple accessory pathways coexisting with a persistent left superior vena cava: a case report.

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.

A Case of Unicentric Castleman Disease with Concomitant Myasthenia Gravis and Persistent Left Superior Vena Cava.

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 draining into the unroofed coronary sinus in a patient with tetralogy of Fallot and coronary sinus orifice atresia.

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.

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.

His bundle lead implantation for leadless pacemaker pacing-induced cardiomyopathy in persistent left superior vena cava.

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.

A rare cause of platypnea-orthodeoxia syndrome in a young female due to persistent left superior vena cava.

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.

Inadvertent Placement of Tunneled Hemodialysis Catheter in Persistent Left Superior Vena Cava.

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.

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.

Prenatal diagnosis of double-outlet right ventricle with tricuspid valve atresia, anomalous pulmonary vein connection, persistent left superior vena cava, and right atrial isomerism.

Double-outlet right ventricle is a conotruncal cardiac disease in which both the aorta and the pulmonary artery predominantly or completely originate from the right ventricle. Here, we report a complex variant double-outlet right ventricle detected in utero and identified on the basis of a segmental approach.

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.

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.

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.