Permanent Pacemaker Implantation in Patients with Persistent Left Superior Vena Cava

A world survey of 34 patients with persistent left superior vena cava who required permanent pacing is reviewed and one case of ours with dominant left superior vena cava is reported. Based on these cases, we conclude that the transvenous, rather than epicardial placement of a permanent pacemaker lead is the procedure of choice in patients with persistent left superior vena cava, with or without coexisting right superior vena cava.     

Sinus Node Dysfunction Associated with Isolated Left Superior Vena Cava

The absence of the right superior vena cava with persistance of the left is a rare venous anomaly, which occurs in 0.05% of general population. This anomaly has been associated with other congenital cardiac defects but rarely with sinus node dysfunction. We report the case of a 52-year-old woman who suffered from symptomatic bradycardia that was associated with supraventricular arrhythmias, and refractory to drugs. Implantation of a pacemaker was necessary and an AAI mode possible because of normal atrioventricular conduction. Insertion of the PM lead was performed via the right cephalic vein, then conducted through the innominate vein into the left persistent superior vena cava, leading to the right atrium via the coronary sinus. The electrode was fixed to the right atrial lateral wall; stimulation threshold was within normal limits. Digital angiography of superior venous inflow confirmed complete absence of the right superior vena cava and described a dilated coronary sinus. No other structural heart disease was present. In accordance with reports in the literature, we found the absence of right superior vena cava complicated by sick sinus syndrome and suggest an etiologic link between these two afflictions. Sinus node function should be studied whenever this venous anomaly is discovered in presence of evocated symptoms.     

Successful Use of Wearable Cardioverter Defibrillator in a Patient with Dextrocardia and Persistent Left Superior Vena Cava

Congenital disorders, such as dextrocardia and persistent left superior vena cava, are rare. However, their presence is often associated with other cardiac anomalies, and may lead to lethal ventricular tachyarrhythmias, which result in sudden cardiac death. Treating patients with these disorders can present a challenge to clinicians, as it may cause technical difficulties during interventional procedures, and more often, altered defibrillation techniques in a setting of prehospital sudden cardiac arrest. This report describes the first case of successful defibrillation therapy delivered by the wearable cardioverter defibrillator to a patient with dextrocardia and persistent left superior vena cava during a ventricular tachycardia arrest.     

Persistent Left Superior Vena Cava Identified During Central Line Placement: A Case Report

INTRODUCTION: A persistent left superior vena cava is found in 0.3-0.5% of the general population and in up to 10% of patients with a congenital cardiac anomaly. It is the most common thoracic venous anomaly and is usually asymptomatic. Being familiar with such anomaly could help clinicians avoid complications during placement of central lines, Swan-Ganz catheters, PICC lines, dialysis catheters, defibrillators, and pacemakers. CASE PRESENTATION: We describe a case of persistent left superior vena cava that was noted after placement of a central line. Mr JJ is a 41 year old African American man who was hospitalized for evaluation and management of alcoholic necrotizing pancreatitis. He required multiple central lines placements. He was noted to have a persistent left superior vena cava that was not recognized initially and thus lead to an unnecessary extra central line placement. DISCUSSION: This anatomic variant may pose iatrogenic risks if it is not recognized by the clinician. A central catheter that tracks down the left mediastinal border may also be in the descending aorta, internal thoracic vein, superior intercostal vein, pericardiophrenic vein, pleura, pericardium, or mediastinum. CONCLUSION: Our case is significant because the patient had two extra central venous catheter placements. This case strongly demonstrates the importance of knowing the thoracic venous anomalies.     

Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava: Not Only an Anatomic Variant

Introduction:

A 71 year old asymptomatic woman came for an echocardiogram because of a left bundle branch block. A much dilated coronary sinus (CS) with an entering large vessel was found along with a mild left ventricular systolic dysfunction. Cardiac Magnetic Resonance (CMR) showed a persistent left superior vena cava (PLSVC), and an absent right superior vena cava (ARSVC). PLSVC drained into the dilated CS. No other cardiac abnormalities were found. Any late Gadolinium enhancement was also not seen. PLSVC and ARSVC are associated with sinus node and conduction tissue maldevelopment and atrial arrhythmias, and thus clinical follow up is indicated.

Conclusion:

CMR is a useful addition to echocardiogram to search for further cardiac abnormalities, and outline the anatomy with precision in doubtful cases.     

Coronary Sinus Ostial Atresia and Persistent Left-Sided Superior Vena Cava: Clinical Significance and Strategies for Cardiac Resynchronization Therapy

A 48-year-old male patient underwent cardiac resynchronization therapy defibrillator implantation, and he was found to have atresia of the coronary sinus ostium with venous drainage occurring via a persistent left-sided superior vena cava, which was connected to the right-sided superior vena cava by the innominate vein. This is a rare benign cardiac anomaly that can pose problems when the coronary sinus needs to be cannulated. To identify the course of the coronary sinus, a coronary angiogram can be performed with attention directed to the venous phase of the angiogram. Although the technical difficulty of coronary sinus cannulation increases, various catheters, wires, and delivery systems can be utilized and this anomaly does not usually prevent successful left ventricular lead placement in cardiac resynchronization therapy via a left-sided superior vena cava approach. There however needs to be consideration regarding caliber of the left-sided superior vena cava being sufficiently large to avoid compromise of venous drainage after lead insertion.     

Hypoxia Caused by Persistent Left Superior Vena Cava Connecting to the Left Atrium A Rare Clinical Entity

We relate the case of a 40-year-old man with a history of premature birth and dextroposition of the heart who presented for an evaluation of persistent hypoxia. An unrevealing pulmonary evaluation and agitated-saline echocardiogram led to cardiac magnetic resonance imaging. This revealed a very unusual finding: a persistent left superior vena cava with insertion into the left atrium and a small connecting vein between the right and left superior venae cavae. The implications, embryology, and pathogenesis of this rare condition are discussed.Key words: Coronary vessel abnormalities/diagnosis, embryology, heart atria/abnormalities, heart defects, congenital/diagnosis, hypoxia, magnetic resonance imaging, middle aged, vena cava, superior/abnormalitiesHypoxia as a clinical entity often has a very clear and easily identifiable cause. Here we describe the case of a patient who presented with resting hypoxia, the cause of which remained unclear after a thorough pulmonary examination and agitated-saline echocardiography. Eventually, a cardiology opinion was sought and cardiac magnetic resonance (CMR) revealed a persistent left superior vena cava (SVC). Surprisingly, this left SVC connected not to the coronary sinus, but led directly to the left atrium; the result was mild, asymptomatic hypoxia.This SVC variant with a left atrial connection is exceedingly rare—found in approximately 0.03% of the population.¹ This case and its accompanying CMR capture the variant in a fantastically clear way.     

Recurrent Cerebral Abscess Secondary to a Persistent Left Superior Vena Cava

Cerebral abscess is a serious neurological condition that is often of unclear etiology. Management is usually medical therapy with or without direct drainage, and when patients have recurrent episodes a structural abnormality should be considered. Persistent left superior vena cava is an uncommon condition in the absence of other forms of congenital heart disease. This venous connection most often enters the right‐sided atrium through the coronary sinus but occasionally can connect directly to the left atrium near the wall between the orifice of the left pulmonary veins and left atrial appendage. This later congenital connection results in systemic venous return entering the left atrium directly. Thus allowing unfiltered, lower saturation blood entering the systemic system. This then places the patient at risk for systemic hypoxemia, paradoxical embolic events, and cerebral abscess. In our case report with recurrent cerebral abscess and a persistent left superior vena cava, we demonstrate when to consider this diagnosis, how to make the diagnosis, and a nonsurgical approach to repair the veno‐atrial shunt.     

Minimally invasive video-assisted thoracoscopic left ventricular epicardial lead implantation for biventricular pacing in a patient with persistent left superior vena cava

Cardiac resynchronization therapy (CRT) by biventricular pacing reduces symptoms and improves left ventricular function in many patients with heart failure due to left ventricular systolic dysfunction and cardiac dyssynchrony. Implantation of the biventricular pacing lead in association with persistent left superior vena cava is technically challenging. We report a successful case of minimally invasive video-assisted thoracoscopic left ventricular epicardial lead implantation for biventricular pacing in a patient with persistent left superior vena cava.     

合并永存左上腔静脉畸形的快速心律失常的经导管射频消融治疗

阐明合并永存左上腔静脉畸形 (PLSVC)的快速心律失常经导管射频消融治疗的方法学和特殊性。自 1 997年 6月至 2 0 0 1年 6月共对 1 3例合并PLSVC的快速心律失常进行了经导管射频消融治疗 ,其中房室结折返性心动过速 (AVNRT) 7例、左侧旁道 3例 ;右侧旁道、房性心动过速 (简称房速 )和室性心动过速 (简称室速 )各 1例。冠状静脉窦标测电极均常规经左锁骨下静脉放置 ,其中 9例进行了冠状静脉窦造影 ,3例左侧旁道中的 2例采用了经主动脉逆行法消融、1例采用穿间隔法在二尖瓣环心房侧消融 ,7例AVNRT中的 5例采用Swartz鞘管支持消融导管实现稳定贴靠。消融成功率 1 0 0 % ,无并发症。结论 :多种快速心律失常可合并PLSVC ,经导管射频消融仍有较高的成功率 ,但是在冠状静脉窦电极放置、冠状静脉窦标测和AVNRT消融的方法学上有一定特殊性。     

Absent Right Superior Vena Cava with Persistent Left Superior Vena Cava Which Drains to Unroofed Coronary Sinus in a Child with Atrioventricular Septal Defect and Cor Triatriatum Sinister: Preop Correct Diagnosis and Successful Surgery in a Single Session

We report a unique case of a 4‐year‐old boy with intermediate‐type atrioventricular septal defect, cor triatriatum sinister, persistent left superior vena cava, unroofed coronary sinus, and absent right superior vena cava. Persistent left vena cava draining into the unroofed coronary sinus was demonstrated easily using the agitated saline‐contrast echocardiography. After conformation with angiographic evaluation, surgery was performed at a single session. Roofing of the coronary sinus with polytetrafluoroethylene patch, mitral cleft repair, tricuspid annuloplasty, atrioventricular defect repair with pericardial patch, and resection of the membrane in the left atrium was succeeded without complication.     

A novel technique for right ventricular lead placement in a patient with a persistent left superior vena cava.

Persistent left superior vena cava is the most common venous anomaly of the thorax. If unrecognized, it could lead to catheter malplacement and even vascular injuries. We describe a novel use of a Worley sheath for the delivery of a right ventricular (RV) endocardial pacing lead in a 65-year-old male with a persistent left superior vena cava. After failed attempts with the standard stylets, use of the Worley sheath aided successful lead deployment. We conclude that when used appropriately, the Worley sheath is a tool that could be helpful in pacing lead placement in patients with persistent left superior vena cava.     

Epicardial CRT-P- and S-ICD Implantation in a Young Patient with Persistent Left Superior Vena Cava

Persistent left superior vena cava is known to be a challenging anatomic abnormality for transvenous cardiac device implantation. In the a case of a young man presenting with dilative cardiomyopathy with severely impaired left ventricular ejection fraction (LVEF) and second-degree atrioventricular block (AV block), cardiac resynchronization therapy (CRT) with defibrillator (CRT-D) implantation was indicated. A transvenous approach was attempted, but placement of the right ventricular lead was not successful due to anatomic abnormalities. Therefore, epicardial CRT leads were implanted via a left mini-thoracotomy. For primary prevention of sudden death, the patient was also fitted with an additional subcutaneous implantable cardioverter defibrillator (S-ICD). Any cross-talk between the devices was ruled out both intraoperatively and by ergometry prior to discharge. The combination of epicardial CRT-P with S?ICD implantation might be a safe and effective alternative in patients with cardiac anatomic abnormalities.     

Arrhythmias in Children Having a Single Left Superior Vena Cava and Minimal Structural Heart Disease

Background: The presence of a single left superior vena cava in the absence of complex congenital heart disease is uncommon, and, in the absence of hemodynamic consequences, it would not be expected to result in cardiovascular signs or symptoms. Single case reports and our anecdotal experience suggested to us that this anomaly is highly associated with cardiac arrhythmias.
Objective: We sought to describe the clinically important arrhythmias in a population of young patients having this anomaly.
Methods: A retrospective chart review was performed from all patients <20 years old and who were determined by echocardiography over an 11-year-period to have a single left superior vena cava and minor or no coexisting congenital heart defects. The prevalence of nonsinus pacemaker, age-corrected sinus rate percentile, and prevalence of brady- or tachyarrhythmias was compared with a control group of patients having bilateral superior vena cavae.
Results: Eight patients having a single left and 55 patients having bilateral superior vena cava(e) were identified. The existence of this anomaly tended to be associated with a lower age-corrected sinus rate percentile (17.5% vs 75%, P = 0.09), and was associated with a higher prevalence of arrhythmias (50% vs 7%, P = 0.014) compared with the control group. In the study group, one patient each had clinically relevant sinus node dysfunction, third-degree AV block, Wolff-Parkinson-White syndrome and atrial fibrillation, and AV nodal reentrant tachycardia.
Conclusion: Even in the absence of symptoms, patients found to have a single left superior vena cava should be monitored long-term for clinically important arrhythmias.     

心房颤动上腔静脉节段性电隔离方法及安全性评估

目的:分析心房颤动(房颤)上腔静脉节段性电隔离的具体手术方法,并评估其安全性.方法:入选2017年11月至2018年9月期间我院阵发性房颤患者50例,患者常规进行肺静脉隔离后,继续行上腔静脉隔离.消融前进行上腔静脉造影,显示上腔静脉与右心房解剖关系,并在CARTO系统运用PentaRay电极导管进行上腔静脉及右心房三维...     

经永存左上腔静脉植入起搏电极体会

目的总结经永存左上腔静脉（PLSVC）植入起搏电极体会。方法总结4例PLSVC植入双腔起搏器的患者。患者出院前及出院后3个月、6个月进行随访,了解起搏器工作状态。结果4例患者术前均常规行经胸超声心动图检查,结果显示冠状静脉窦开口扩大,提示PLSVC存在可能。第1例患者因术前发现PLSVC可能,首先穿刺右侧锁骨下静脉;造影显示右上腔静脉缺如,右侧锁骨下静脉汇入PLSVC。第2、3例患者因双腔起搏器植入前反复出现心动过缓相关症状,在术前经右侧锁骨下静脉植人临时起搏器,术中造影显示PLSVC与右侧上腔静脉无交通。第4例患者在置入导丝时直接经PLSVC进入右心房。患者术后3个月、6个月常规行起搏器程控并调整起搏器出厂设置参数,此后每6—12个月行起搏器程控,程控时测量心房电极和心室电极的起搏阈值、感知和阻抗。均在满意范围。第1例患者两次随访时心律均为窦性心律,心房电极感知大于2mV,起搏阈值小于1V,阻抗小于1000Q。结论PLSVC能够顺利完成起搏电极植入。     

Unusual Combination of Holt‐Oram Syndrome and Persistent Left Superior Vena Cava

Holt‐Oram (HO) is a syndrome characterized by congenital cardiovascular malformations, specifically atrial and ventricular septal defects, and skeletal abnormalities of the upper limbs bones. Associations of HO cardiac disorders with other congenital cardiac malformations, specifically persistent left superior vena cava (PLSVC) are rarely reported and its real incidence is unknown. We present a case of this unusual combination in a patient undergoing cardiac resynchronization therapy (CRT) device implant. Methods and Results. A 63‐year‐old male with HO and a history of repaired atrial septal defect was presented for implantable cardioverter defibrillator (ICD) upgrade to CRT. The old implant was located in the right prepectoral area. The old device pocket in the right was accessed and a venous access to the right subclavian vein was obtained. The coronary sinus (CS) was easily cannulated and a long sheath advanced into the CS. A contrast injection revealed an unusually big‐sized CS, with a diameter 2.5 times the fully deployed balloon. A 0.035 wire was advanced retrograde reaching the confluence of the innominate and left subclavian veins. The outer sheath was advanced to this location and contrast venography through the sheath allowed visualization of the left jugular and subclavian veins and visualization of the PLSVC draining into the CS. No target veins for lead implant were identified. The patient was referred for surgical implant of an epicardial lead. Transesophageal echocardiogram showed a CS identified as an unusually big vascular structure located between the left atrium and the left atrial appendage. Conclusion. We report an uncommon association of HO and PLSVC. This association was only reported twice in the past and this is the first one that constitutes a casual finding during the attempt of CRT device implant. This is a combination that may complicate a device implant and recognition of it in advance may avoid performing potentially unsuccessful procedures.     

Transvenous biventricular pacing for cardiac resynchronization therapy in patients with persistent left superior vena cava and right superior vena cava atresia

Biventricular pacing for cardiac resynchronization therapy is an effective adjunctive therapy for the treatment of symptomatic moderate and severe congestive heart failure. However, experience with transvenous cardiac resynchronization therapy in patients who have both persistent left superior vena cava and right superior vena cava atresia is extremely limited. We successfully performed cardiac resynchronization therapy in 2 patients who had persistent left superior vena cava, right superior vena cava atresia, and congestive heart failure. Our 2 cases demonstrate the possibility of a total transvenous approach for left ventricular pacing despite the presence of serious cardiac venous anomalies. This approach enables clinicians to avoid the riskier epicardial lead placement, which requires a thoracotomy under general anesthesia.     

Single Atrium Associated with Persistent Left Superior Vena Cava in Asymptomatic Adult: Case Report and Review of Literature

We report a rare case of an adult patient with single atrium without the characteristics of an endocardial cushion defect, associated with persistent left superior vena cava draining to the left side of the atrium. The patient was reasonably active and asymptomatic till the third decade of his life when the cardiac anomaly was discovered and surgically corrected.     

Persistent Left Superior Vena Cava Draining into Left Atrium as a Cause of Persistent Systemic Desaturation after Surgery

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