Ostium primum atrial septal defect with persistent left superior vena cava opening into unroofed coronary sinus—A rare entity

Raghib syndrome is a rare developmental complex consisting of termination of the left superior vena cava in the left atrium, absence of the coronary sinus, and an atrial septal defect commonly located at the posterior‐inferior angle of the atrial septum. This complex was considered unique to Raghib syndrome; however, cases with a normal atrial septum have been reported where the orifice of the unroofed coronary sinus functions as the inter‐atrial communication. Our patient demonstrated an isolated persistent left superior vena cava draining into the left atrium through unroofed coronary sinus and presence of ostium primum atrial septal defect.     

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.     

Isolated persistent left-sided superior vena cava,giant coronary sinus,atrial tachycardia and heart failure in a child

Persistence of a left-sided superior vena cava (PLSVC) with absent right superior vena cava (isolated PLSVC) is a very rare venous malformation and commonly associated with congenital heart disease or alterations of the cardiac situs. We describe an unusual case of a young boy presenting with persistent atrial tachycardia and congestive heart failure. He was detected to have unexplained grossly dilated right atrium, right ventricle with systolic dysfunction and a giant coronary sinus (CS). The dilated CS closely mimicked a pseudo cor-triatriatum on echocardiography. Contrast echocardiography from both arms revealed opacification of the CS before the right atrium. Bilateral upper limb venography confirmed the presence of absent right SVC and isolated persistent left SVC draining into the giant coronary sinus.     

Coronary sinus ostial atresia with thrombus: a case report

An 82-year-old woman presented with mitral regurgitation and atrial fibrillation. She was hospitalized for further examination of cardiac thrombus detected by transthoracic echocardiography. No definitive diagnosis could be made based on computed tomography and magnetic resonance imaging. Intravenous contrast echocardiography suggested a coronary sinus thrombus. Coronary angiography showed coronary sinus ostial atresia. Multislice computed tomography confirmed the presence of thrombus in the enlarged coronary sinus with coronary sinus ostial atresia and persistent left superior vena cava. Persistent left superior vena cava is present in 3% of patients with congenital heart disease. Persistent left superior vena cava is detected by chance during cardiac catheterization for other conditions in patients without cardiac malformations. The present case was detected at an advanced age, and in the absence of prior symptoms.     

Persistent left superior vena cava,absent right superior vena cava and coronary-bronchial fistula: The good,the bad and the ugly (case report and review of literature)

A 62-year-old Caucasian male presented with syncope during casual daily activity without preceding prodromes. During ECG Holter monitoring, we observed numerous asystolic pauses lasting >4 s due to sino-atrial blockade and sinus bradycardia. During pacemaker implantation, persistent left superior vena cava with agenesis of the right superior vena cava was diagnosed. Unproblematic placement of atrial lead was followed by challenging placement of the right ventricular lead. Anterior position with a sharp angulation to the right ventricular wall was achieved with excellent stimulation parameters. Transesophageal echocardiography confirmed the diagnosis of persistent left superior vena cava with agenesis of right superior vena cava. Moreover, selective coronary angiography showed connection between right coronary artery branch and bronchial vessel. To the best of our knowledge, we are the first to describe a combination of persistent left superior vena cava with absent right superior vena cava, coronary-bronchial fistula and conduction abnormality with the necessity of device implantation.     

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.     

Absent right superior vena cava with left superior vena cava draining to an unroofed coronary sinus

We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.     

Echocardiography of the coronary sinus in adults

The coronary sinus (CS) is a small tubular structure just above the posterior left atrioventricular junction. The CS can be imaged in several different echocardiographic views. Using zoom M-mode recordings of the CS in apical two-chamber view, CS caliber can be sharply imaged and easily measured during different phases of the cardiac cycle. We have recently shown that the CS narrows during atrial contraction in persons with sinus rhythm, but does not narrow at all if atrial fibrillation is present. Attenuation of CS narrowing occurs in patients with congestive heart failure and inferior vena cava plethora. Maximal CS caliber occurs during ventricular systole. Patients with poor left ventricular systolic function show mild CS dilatation. Greater CS dilatation is present in patients with persistent left superior vena cava, and huge dilatation when this anomaly is accompanied by absence of a right superior vena cava. Injection of sonicated saline into a left and then a right arm vein is diagnostically useful in confirming these two venous anomalies. Pulsed-wave Doppler of the CS can be recorded in the parasternal right heart inflow view. From this and from the CS cross-section area it may be possible to estimate coronary blood flow.     

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.     

Absent right and persistent left superior vena cava

A patient is described presenting with atrial fibrillation. A dilated coronary sinus was found due to the presence of a persistent left superior caval vein. Absence of the right superior caval vein was suspected with contrast injection through an i.v. line in the right arm, and was confirmed with phlebography. No associated cardiac anomalies were found. Persistent left superior vena cava is a common anomaly, although simultaneous complete absence of the right superior vena cava is rare. The incidence, embryology, diagnosis and importance of this anomaly is discussed.     

Cavernous haemangioma in the coronary sinus

A 58 year old man with a history of cerebral infarction was admitted to hospital with chest discomfort and dyspnoea. He had no history of precordial chest discomfort. Angiography and left ventriculography showed that coronary fistulas connected the coronary sinus with the left circumflex and right coronary arteries. His coronary sinus did not communicate with the right atrium, draining instead into a persistent left superior vena cava. Angiography showed a mass, suspected to be a thrombus, in the coronary sinus. Transoesophageal echocardiography confirmed the presence of a mass in the atrioventricular groove. The mass was removed at surgery and proved to be a cavernous haemangioma.

Keywords: coronary fistulas;  persistent left superior vena cava;  cavernous haemangioma;  coronary sinus     

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.     

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.     

Transvenous cardioverter-defibrillator implantation with a double coil lead via persistent left superior vena cava.

A dual-coil defibrillation lead was inserted in a 64-year-old male through a persistent left superior vena cava draining into the coronary sinus. The lead, connected to a cardioverter-defibrillator (ICD) implanted in the left pectoral area, was looped in the right atrium positioning the proximal and distal lead coils in the coronary sinus and right ventricular outflow track respectively and resulting in a low and stable defibrillation threshold. Because of its relative ease and effectiveness, this procedure may be recommended in patients with persistent left superior vena cava requiring an ICD implant.     

Diagnosis of a complete atrioventricular canal with infundibular pulmonary stenosis and left superior vena cava by 2-dimensional contrast echocardiography. Apropos of a surgically treated case

The case of an eight year old child with complete atrioventricular canal, pulmonary infundibular stenosis and persistent left superior vena cava draining into the coronary sinus is reported. Two-dimensional echocardiography with injection of contrast in a left arm vein gave a precise and complete diagnosis of the malformations before catheterisation and angiography. The complete atrioventricular canal was demonstrated by apical four-chamber views. The pulmonary infundibular stenosis was visualised by a short axis subcostal view. Contrast echocardiography in the apical four-chamber view showed a right-to-left shunt at atrial level at the site of the ostium primum and a right-to-left shunt at ventricular level just below the hemivalve. The left superior vena cava was detected by a short axis suprasternal view which visualised its vertical trajectory as far as the coronary sinus. The lesions were confirmed at surgery, and a complete repair was performed.     

A complex anomaly of systemic and pulmonary venous return associated with sinus venosus atrial septal defect

This report describes a rare case of anomalous systemic and pulmonary venous return that was surgically corrected. It consisted of left inferior vena cava with hemiazygous continuation into the persistent left superior vena cava, partial anomalous pulmonary venous return from the right lung and sinus venosus atrial septal defect.     

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.     

Double superior vena cava with drainage of the right superior vena cava into the left auricle. Presentation as a cerebral abscess in an adult

A 44 year old man presented with a cerebral abscess, the location of which suggested a septicaemic origin. Although the patient was not cyanosed, a cardiological work-up was requested to exclude a right-to-left shunt. This showed a double abnormality of the systemic venous drainage: presence of an abnormal left superior vena cava draining into the coronary sinus and of a right superior vena cava draining into the left atrium. These two vena cava intercommunicated by anastomoses. Angiography in the right superior vena cava after occlusion by balloon catheter at its junction with the left atrium showed flow from the right to the left superior vena cava and to the azygos system. Simple ligature of the right superior vena cava was therefore performed to prevent recurrence of cerebral abscess. This case is rare and of interest because of the presence of two superior vena cavae, one on the right draining into the left atrium and the other on the left draining into the coronary sinus, with anastomoses between the two superior vena cavae. This double abnormality of systemic venous drainage explains the absence of cyanosis and therefore the relatively late detection of this malformation.     

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.     

A case of absent right superior vena cava with persistent left superior vena cava: Cross-sectional echocardiographic diagnosis

Summary We report a case of absence of the right superior vena cava. Hitherto, the findings in this condition have only been obtained using invasive methods; this report is the first in which the diagnosis was made by means of echocardiography. The findings include a markedly enlarged coronary sinus draining into the right atrium and a specific finding of contrast echocardiography in which contrast material injected into the right antecubital vein can be seen in the enlarged coronary sinus. The contrast echo flows into the right atrium from the coronary sinusonly. The size of the coronary sinus was far beyond the range (91±38 mm²/m²) in patients with persistent left superior vena cava with the presence of the right superior vena cava.