Left superior vena cava: a simple diagnosis by transthoracic echocardiography. A case report

Persistent left superior vena cava is an anomaly of the systemic venous return occurring in 0.5% of the general population. We report the case of a patient with an incidental diagnosis made during a dyspnea while he had chronic pulmonary disease. The diagnosis was suspected by the presence of a markedly dilated coronary sinus and confirmed by a simple contrast injection into the left antecubital vein. Transesophageal echocardiography and magnetic resonance imaging confirmed the existence of 2 superior vena cava with the left superior vena cava draining into the coronary sinus. This congenital anomaly is of minimal hemodynamic significance when isolated. The diagnosis can be useful for placement of central catheters from left superior approach.     

Persistence of the left superior vena cava discovered during the implantation of a hemodialysis catheter

A persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. It is a persistent remnant of the left anterior cardinal vein that usually disappears in early embryological development as a result of compression between the left atrium and the hilum of the left lung. If it is not associated with other congenital cardiac anomalies it is usually asymptomatic but has important clinical implications in some situations. In this article, we describe a patient with bilateral SVC identified on a chest radiograph by a haemodialysis central venous catheter passing through it.     

Placement of a pulmonary artery catheter via a previously unrecognized persistent left superior vena cava

This case report describes a patient with persistent left superior vena cava (LSVC) as discovered by difficult placement of a pulmonary artery catheter via the left subclavian vein. After positioning in wedge position, chest x-ray showed a catheter route suggestive of persistent LSVC. Since this abnormality may yield potential clinical complications, this possibility should be considered in every difficult central venous access.     

Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance

Persistent left superior vena cava (PLSVC) is a very rare and yet the most commonly described thoracic venous anomaly in medical literature. It has a 10-fold higher incidence with congenital heart disease. PLSVC often becomes apparent when an unknown PLSVC is incidentally discovered during central venous line placement, intracardiac electrode/pacemaker placement or cardiopulmonary bypass, where it may cause technical difficulties and life-threatening complications. PLSVC is also associated with disturbances of cardiac impulse formation and conduction including varying degrees of heart blocks, supraventricular arrhythmias and Wolff Parkinson White syndrome. We describe the case of an 86-year-old male with a history of coronary artery disease and chronic atrial fibrillation who presented with worsening dyspnea and syncopal episodes. An ECG was consistent with complete heart block. During lead placement for the pacemaker, a left subclavian approach was unsuccessful. A left venogram was performed through the brachial vein that demonstrated a left superior vena cava. The diagnosis was confirmed with echocardiography using a bubble study and also a chest CT. The anatomy was unique as there was anomalous left hepatic vein drainage into the right atrium. The case provides insight into the diagnostic modalities and clinical considerations of this unusual thoracic venous anomaly.     

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.     

Implantation eines Resynchronisationssystems bei persistierender oberer Hohlvene

We report an implantation of a cardiac re-synchronization system in a patient with persistent left superior vena cava. This anomaly occurs in 0.3 to 0.5% of healthy individuals and remains usually asymptomatic. Variations of the superior vena cava should be considered in venous catheterization and other procedures such as implantation of pacemaker and ICD systems as well as port catheter insertion. In re-synchronization systems, persistent left superior vena cava can be an obstacle for cannulation of the coronary sinus and placement of a transvenous left ventricular lead.     

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.     

Two cases of left superior vena cava draining directly to a left atrium with a normal coronary sinus.

The most common variation in the thoracic systemic venous system is a persistent left superior vena cava draining to a coronary sinus. A rare anomaly is a persistent left superior vena cava connecting directly to the left atrium. In this situation it is believed that the coronary sinus must be absent. This report describes two cases of a persistent left superior vena cava draining to a left atrium with a normal coronary sinus.     

Two cases of left superior vena cava draining directly to a left atrium with a normal coronary sinus

The most common variation in the thoracic systemic venous system is a persistent left superior vena cava draining to a coronary sinus. A rare anomaly is a persistent left superior vena cava connecting directly to the left atrium. In this situation it is believed that the coronary sinus must be absent. This report describes two cases of a persistent left superior vena cava draining to a left atrium with a normal coronary sinus.     

Venobronchial fistula: a complication associated with central venous catheterization for chemotherapy

We report a case in which a venobronchial fistula developed 4 months after insertion of a silicone rubber chronic central venous catheter for chemotherapy administration. In retrospect, the tip of the catheter was in the azygous vein rather than in the superior vena cava. The suboptimal position of the catheter tip, in combination with the infusion of sclerosing chemotherapeutic agents and hyperalimentation solution, may have predisposed the patient to this complication. Accurate localization of the tip of central venous catheters at the time of placement should minimize catheter-related complications, including the rare complication of venobronchial fistula. After placement of chronic central venous catheters, biplanar chest X-rays should be obtained to determine catheter tip location prior to the instillation of chemotherapeutic agents. Any residual questions concerning the catheter tip location after biplanar X-rays should be addressed by prompt venography through the catheter.     

Persistent left superior vena cava and pacemaker implantation

Our study group read with interest the paper from Vijayvergiya et al describing the implantation of an implantable cardioverter-defibrillator lead in the presence of the persistence of the left superior vena cava.The issue of the identification a persistent left superior vena cava is of paramount importance in interventional cardiology,being the most common venous anomaly of the thoracic distribution,and because it may create some problem to any physician while performing a pacemaker lead implantation.In our letter we underscore the specific issues related to pacemaker implantation while encountering a persistent left superior vena cava(and maybe the absence of the right vena cava)and the workup that should be performed to obtain the preoperative diagnosis of the venous anomaly.More specifically,we consider avoiding any kind of defibrillator lead implantation through the coronary sinus for safety issues,and underscore the straightforward transthoracic ultrasound approach to identify the left superior vena cava.     

Giant coronary sinus and absent right superior vena cava

Although persistent left superior vena cava (PLSVC) is rare, it is the most common thoracic venous anomaly. The prevalence of PLSVC is 0.3 % in a general population and 10 % in patients with congenital heart disease. Diagnosis of PLSVC is usually incidental during cardiovascular imaging or surgery. Although PLSVC is usually not associated with any negative hemodynamic effect, it is important to be aware of its existence, since it may cause problems in central venous catheterization, pacemaker implantation and cardiopulmonary bypass. The overall proportion of absent right superior vena cava in patients with PLSVC is approximately 20 %. If PLSVC is associated with absence of the right superior vena cava, the coronary sinus may become gigantic due to excessive inflow. Herein, we report a case of PLSVC complicated by the absence of a right superior vena cava resulting in a giant coronary sinus.     

Unusual venacaval anomalies in a patient with atrial septal defect

An unusual systemic venous drainage pattern was found in a 30-year-old man with ostium secundum atrial septal defect and pulmonary stenosis. He had the rare association of absent right superior vena cava, persistent left superior vena cava draining into the coronary sinus, and a left-sided inferior vena cava draining into a left superior vena cava through the hemiazygous vein.     

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.     

A case report of anomalous left brachiocephalic vein

An anomalous case of a left brachiocephalic vein passing behind the ascending aorta was observed in a 49-year Japanese man. This is known as anomalous left brachiocephalic vein. The anomalous left brachiocephalic vein descended along the left mediastinum in a position identical to that of a persistent left superior vena cava, so the diagnosis of this venous abnormally require carefully considered. Many cases of anomalous left brachiocephalic vein have been reported based on autopsy findings, but this venous anomaly was recently demonstrated by ultrasonography, CT, and MRI.     

Absence of the left brachiocephalic vein with venous return through the left superior intercostal vein: CT findings

Asymptomatic congenital thoracic venous anomalies are becoming clinically more relevant with the increasing utilization of minimally invasive surgical vascular procedures, such as left-sided implantable cardioverter defibrillator implantation. The purpose of this report is to describe the computed tomography findings of the congenital absence of the left brachiocephalic vein in a patient with no evidence of congenital cardiovascular disease and no prior history of central venous instrumentation. In this patient, the left internal jugular and the left subclavian veins drain via the left superior intercostal vein, the accessory hemiazygous, the hemiazygous, and the azygous vein into the right brachiocephalic vein to form the superior vena cava. The clinical significance and possible embryogenesis of this anomaly are discussed.     

Surgical correction of isolated persistent left superior vena cava draining to left atrium in a neonate.

A one day old baby who presented with cyanosis was later shown to have an isolated persistent left superior vena cava draining into the left atrium with hypoplastic right superior vena cava. This extremely rare anomaly was diagnosed by cardiac catheterisation at one week. Surgical correction at two weeks, by anastomosis of the innominate vein to the right atrium and by ligation of the left superior vena cava at its junction with the left atrium, was successful. This patient is the youngest case in which successful surgical correction of this isolated anomaly has been reported.     

Surgical correction of isolated persistent left superior vena cava draining to left atrium in a neonate

A one day old baby who presented with cyanosis was later shown to have an isolated persistent left superior vena cava draining into the left atrium with hypoplastic right superior vena cava. This extremely rare anomaly was diagnosed by cardiac catheterisation at one week. Surgical correction at two weeks, by anastomosis of the innominate vein to the right atrium and by ligation of the left superior vena cava at its junction with the left atrium, was successful. This patient is the youngest case in which successful surgical correction of this isolated anomaly has been reported.     

Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device.

A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.     

Superior vena cava anomalies in the generation of angina pectoris: a report of two cases

Superior vena cava anomalies are rare occurrences caused by variations in the development of the embryonic venous system. Persistent left superior vena cavae are the most common congenital aberrations in the thoracic venous system, with an incidence of 0.3-0.5%, but their association in the absence of a right superior vena cava is extremely rare and scarcely reported. We report two case studies describing persistent left superior vena cavae found with and without a right superior vena cava in patients presenting with chest pain. A discussion regarding superior vena cava abnormalities as well as the etiology, associations, and diagnosis of these unusual entities follows.