Coronary sinus atresia and persistent left superior vena cava with the presence of thrombus complicating implantation of a left ventricular pacing lead.

A 68-year-old male with heart failure and a suitable candidate for resynchronization therapy was referred to our hospital because of a failed coronary sinus (CS) lead implant. Catheterization of the CS initially also failed in our department but a left coronary angiogram revealed atresia of the CS and drainage of the coronary venous system via a persistent left superior vena cava (PLSVC). Implantation of a CS lead through the PLSVC could be accomplished after a selective angiogram, even in spite of the presence of a large thrombus at the junction of PLSVC and CS.     

Creation of a coronary sinus to atrial communication in coronary sinus ostial atresia improves cardiac function after Fontan.

Coronary sinus ostial atresia is a rare anatomic condition which is occasionally associated with drainage of coronary veins via a persistent left-sided superior vena cava. This anatomic condition is normally clinically insignificant. However, when the systemic venous pressure is higher than that of the atrium, cardiac function can be impaired. We report our experience with a young adult who underwent a Fontan due to tricuspid atresia. The patient presented to our institution with poor systolic and diastolic ventricular function. A catheterization revealed an atretic coronary sinus ostium, and the coronary veins drained via a persistent left-sided superior vena cava. Creation of a communication from coronary sinus to the common atrium resulted in a significant decrease in coronary venous pressures. The patient has experienced improvement following the interventiona catheterization.     

Technical failure to perform cardiac resynchronization therapy: use of cardiac magnetic resonance imaging techniques to clarify a left-sided superior vena cava and coronary sinus morphology

The most common reason for failure to implant a left ventricular lead to deliver cardiac resynchronization therapy is the presence of unfavourable coronary venous anatomy. The present report illustrates the use of cardiac magnetic resonance imaging to delineate the anatomy of a left-sided superior vena cava in two patients in whom permanent cardiac pacing was unattainable.     

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.     

Permanent pacemaker implantation via left superior vena cava without communication with the coronary sinus

The persistence of the left superior vena cava is a rare venous anomaly and usually does not produce hemodynamic disturbances. Left sided cardiac device implantation has increased the awareness of this rare anomaly. In most cases, left superior vena cava connects to the right heart via coronary sinus. We describe a rare case of successful permanent pacemaker implantation via left superior vena cava-accessory hemiazygos-hemiazygos-inferior vena caval communication.     

Fetal echocardiography in detecting anomalous pulmonary venous connection: four false positive cases.

Prenatal detection of congenital heart disease is possible from the 16th week of pregnancy, the ideal time being the mid-trimester, when most cardiac abnormalities can be detected. However, identification of anomalous pulmonary venous connection is difficult before birth and the sensitivity of fetal echocardiography in detecting this anomaly is low. Four cases are reported in which fetal echocardiographic findings obtained during the third trimester of pregnancy were highly suggestive of anomalous pulmonary venous connection. Right ventricular and atrial dominance associated with an enlarged coronary sinus or dilated superior vena cava were identified and considered to be indirect markers of the anomaly. No other cardiac anomaly was detectable. In all cases right ventricular and atrial dominance with dilated coronary sinus or superior vena cava were confirmed after birth despite the presence of normal pulmonary venous connections. These results confirm that the prenatal detection of this condition is difficult and should be based on the direct visualisation of anomalous pulmonary venous connections. The sole detection of indirect signs, such as right atrial and ventricular dominance with or without a dilated coronary sinus, superior vena cava, or inferior vena cava, does not warrant the diagnosis of anomalous pulmonary venous connection. Since the pulmonary venous flow in the human fetus is not as small as is commonly assumed, an anomalous drainage should be detectable when present and therefore should be specifically sought if the anomaly is suspected. The reasons for the presence of such transient cardiac anomalies remain obscure, but they might be related to functional or morphological rearrangement of the heart during fetal and perinatal life.     

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.     

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.     

Inability to Cannulate the Coronary Sinus in Patients with Supraventricular Arrhythmias: Congenital and Acquired Coronary Sinus Atresia

We report 4 cases of congenital and acquired coronary sinus ostial atresia incidentally found during electrophysiologic assessment for supraventricular arrhythmias. Congenital variants consisted of coronary sinus drainage predominantly via persistent left superior vena cavae and partial coronary sinus unroofing into the left atrium. The acquired variant was inadvertently produced during surgery for cor triatriatum. A variety of electrophysiologic substrates including right and left-sided accessory pathways and both typical and atypical AV nodal reciprocating tachycardia were identified. Approaches to imaging and accessing the coronary sinus when the os cannot be cannulated are discussed, including a search for right atrial accessory venous collaterals, venography to rule-out a persistent left superior vena cava, and coronary angiography.     

Partial anomalous venous return associated with intact atrial septum and persistent left superior vena cava: a case report and literature review

Left-sided partial anomalous pulmonary venous return (PAPVR) with an intact atrial septum is a rare cardiovascular anomaly. This report deals with the case of a 22-year-old woman who was referred to our Institution because of resting palpitation and exertional dyspnea. Two-dimensional echocardiography revealed right heart volume overload and a dilated coronary sinus. A left-sided PAPVR draining into a persistent left superior vena cava which, in turn, entered a dilated coronary sinus, was correctly detected by magnetic resonance imaging. Diagnosis was confirmed by cardiac catheterization. The literature on this subject is reviewed.     

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.     

Anomalies of cardiac venous drainage associated with abnormalities of cardiac conduction system.

The embryological development of the superior vena cava (SVC) is complex. If the left common cardinal vein fails to occlude it can, along with the left duct of Cuvier form a left SVC, which frequently drains into the coronary sinus. This may result in abnormalities in the anatomy of this structure. A persistent left SVC occurs in 0.5% of the normal population, and 3% to 4.3% of patients with congenital heart anomalies. The pacemaking tissue of the heart is derived from two sites near the progenitors of the superior vena cava. The right-sided site forms the sinoatrial node, the left-sided site is normally carried down to an area near the coronary sinus. Out of 300 patients with cardiac rhythm abnormalities, who have undergone electrophysiological studies (EPS), or permanent pacemaker insertion (PPI), we identified 12 patients with cardiac conduction abnormalities and anomalies of venous drainage. Anomalies of the coronary sinus may be associated with abnormalities of the conduction system of the heart. This may be due to the close proximity of the coronary sinus to the final position of the left-sided primitive pacemaking tissue. In our series of 300 patients, 4% had an associated left SVC, a similar incidence to that found in previous studies of congenital heart disease.     

Mitral valve replacement in patient with absent right superior vena cava, pectus excavatum, Marfan syndrome and severe mitral regurgitation

Combination of persistent left superior vena cava (PLSVC) draining into right atrium via the coronary sinus with a normal right superior vena cava (RSVC) is the most common systemic venous anomaly in visceroatrial situs solitus. On the other hand, a PLSVC in absence of RSVC is an extremely rare anomaly. Cardiovascular abnormalities are frequently encountered in patients with Marfan's syndrome but absence of RSVC with PLSVC is not reported in these patients. We report on a 52-year-old man with Marfan syndrome, an absence of right superior vena cava, severe pectus excavatum and severe mitral regurgitation who underwent successful mitral valve replacement. CT scan chest revealed marked cardiac deviation to the left associated with severe pectus excavatum, absent right superior vena cava and persistent left superior vena cava. During surgery replacement of mitral valve was performed through midsternotomy successfully.     

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.     

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.     

Reopening of a persistent left superior vena cava in the early postoperative period following bidirectional cavopulmonary anastomosis--treatment by coil embolization]

Diagnosis of systemic venous drainage is mandatory for patients with congenital heart disease planned for cavopulmonary anastomosis or any Fontan-type palliation. Preexisting venous channels as the persistent left superior vena cava are common in cardiac anomalies and may lead to postoperative deterioration and cyanosis. We describe a 6 month old boy with a complex imbalanced atrioventricular septal defect who developed cyanosis in the very early postoperative period, following bidirectional cavopulmonary anastomosis. It was caused by ineffective lung perfusion due to a reopened persistent left superior vena cava with drainage to the coronary sinus.He underwent coil embolization of the persistent left superior vena cava with retrievable coils and cyanosis improved. Coil embolization is an effective alternative to secondary surgery, especially for hemodynamically compromised patients in the postoperative period.     

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.     

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.     

Left-sided inferior vena cava draining into the coronary sinus via persistent left superior vena cava: Case report and review of the literature

A case of a left-sided Inferior vena cava draining into the coronary sinus via a persistent left superior vena cava is described and the pertinent literature reviewed.