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.     

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.     

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.     

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.     

Echocardiographic features of total anomalous pulmonary venous drainage into the coronary sinus

Echocardiograms were obtained from five infants with total anomalous pulmonary venous drainage to the coronary sinus or the portal system or the superior vena cava and from one child with a secundum atrial septal defect and a large coronary sinus due to persistence of the left superior vena cava. The results demonstrate that an enlarged coronary sinus is positioned consistently posterior to the left atrium in approximately the same horizontal plane as the aortic valve. Echocardiographically the coronary sinus can be located as an echo complex behind the left atrium by using the aortic root as a reference point. The echo complex can be differentiated from the other spurious echoes in the left atrium by its characteristic phasic motions. The additional demonstration of the enlarged common pulmonary venous chamber behind the right atrium confirms the diagnosis of anomalous pulmonary venous drainage to the coronary sinus. For other types of anomalous pulmonary venous return, anatomic diagnosis with single crystal M mode echocardiography is not always possible because of the positional variability of the common pulmonary venous chamber in relation to the left atrium.     

Coronary sinus long sheath catheter system: A new device for transfemoral coronary sinus catheterization

Summary Coronary sinus catheterization requires an approach into the right atrium via the superior vena cava. This study contains information regarding a coronary sinus long sheath catheter (CS sheath) system, a new device for cannulation of the coronary sinus through the femoral approach. This method was successful in 96.9% of the cases attempted. Furthermore, cannulation using the CS sheath allowed us not only to insert several catheters into the coronary sinus for clinical use and investigation, but also to access the great and small cardiac veins easily and selectively.     

Atresia of the right atrial orifice of the coronary sinus with persistent left superior vena cava: a case report

A 39-year-old woman presented with atresia of the right atrial orifice of the coronary sinus with a persistent left superior vena cava detected at cardiac catheterization. She was admitted with frequent episodes of angina at rest and on exertion. Coronary angiography, including spasm provocation test, yielded normal results. However, left coronary arteriography demonstrated a dilated coronary sinus and a persistent left superior vena cava draining into the innominate vein. The contrast medium leaked slightly into the right atrial cavity through the obstructed orifice of the coronary sinus. Atresia of the coronary sinus orifice is a rare malformation usually found at autopsy. Only 3 cases have been reported in Japan. This is the first adult Japanese case detected when the patient was still alive.     

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.     

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.     

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.     

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.     

Childhood angina due to congenital atresia of the coronary sinus

A young child with exertional chest pain, and an electrocardiographic pattern suggesting reversible ischaemia of the anterior ventricular wall documented by Cardiolyte stress-testing, underwent cardiac catheterization and selective coronary angiography. Although the coronary arteries were entirely normal, the recirculation phase demonstrated marked dilation of the coronary sinus, with atresia of its mouth. At surgery, the patient was confirmed to have muscular atresia at the mouth of the coronary sinus, and underwent unroofing of the coronary sinus to the left atrium, with ligation of a persistent left superior caval vein. Post-operatively, the patient continued to have persistent chest pain, albeit without inducible ischaemia on stress-testing.     

Coronary sinus mapping through a persistent left superior vena cava in Wolff-Parkinson-White syndrome

Persistent left superior vena cava is not an uncommon finding in patients undergoing evaluation for preexcitation syndromes. In such patients, this anatomical configuration might be used advantageously for mapping and ablation of a left-sided accessory pathway during electrophysiologic studies. We successfully used this mapping approach in a 16-year-old boy undergoing evaluation for Wolff-Parkinson-White syndrome. The patient was found to have a persistent left superior vena cava confluent with the coronary sinus. During electrophysiologic studies, mapping of the left-sided accessory pathway was facilitated by retrograde entry into the coronary sinus through the persistent left superior vena cava. Mapping was also performed in the conventional manner, yielding identical results and thus validating this new technique.     

Double superior vena cava: right connected to left atrium and left to coronary sinus

Left persistent superior vena cava connected to coronary sinus may coexist with right superior vena cava connected to left atrium. Surgical correction is not mandatory if there is no cyanosis due to a big interjugular bridging vein.     

Retrograde non trans‐septal balloon mitral valvotomy in mitral stenosis with interrupted inferior vena cava,left superior vena cava,and hugely dilated coronary sinus

A 22‐year‐old woman with severe mitral stenosis was referred to us for further evaluation and management. She was found to have severe mitral stenosis, severe tricuspid regurgitation with dilated right atrium and right ventricle with persistent left superior vena cava and hugely dilated coronary sinus. Valve was suitable for balloon mitral valvotomy. Cardiac catheterization showed interrupted inferior vena cava with azygos continuation to right atrium and large left superior vena cava draining to coronary sinus which was very much dilated. Right trans‐jugular approach was tried for balloon mitral valvotomy, but was unsuccessful due to a very large right atrium and coronary sinus. Retrograde non trans‐septal approach was used and balloon valvotomy was done successfully using a 24 mm × 40 mm TYSHAK balloon without any major complication. Reduction in the transmitral pressure gradient on cardiac catheterization data and transthoracic echocardiography confirmed successful procedure. Balloon mitral valvotomy can be done successfully in patients with the above unusual cardiac anatomy with no major procedural complications. © 2015 Wiley Periodicals, Inc.     

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     

Echocardiographic aspects of persistence of the right valve of the venous sinus

The aim of this work is to demonstrate the possibility to identify persistent right sinus venous valve and to differentiate it from other right sided heart pathology by two dimensional echocardiography. We report the echocardiographic findings observed in three out of 215 paediatric patients we examined for clinically suspected congenital heart disease in 12 months period. The first patient was a 24 hours old newborn with transient pulmonary hypertension; the second one was a 6 months old child with pulmonary atresia, severe right ventricular hypertrophy, atrial septal defect and patent ductus arteriosus; the third patient, aged 6 months, had atrial septal defect and mild pulmonary stenosis. In all three patients a particular linear structure was seen, as a membrane that crossed the right atrium from the orifice of the inferior vena cava towards the atrial septum. This structure was identified as persistent right sinus venosus valve according to its morphology, its position into the right atrium and its connection to the atrial septum. The incidence of this echocardiographic finding was 1.4% in our series. In the first case contrast echocardiography from the inferior vena cava demonstrated the deviation of blood flow caused by the persistent sinus valve, although no intra-atrial pressure gradient was shown at cardiac catheterization. In the second patient who died during the operative procedure for making a systemic-pulmonary anastomosis, post mortem examination showed a membranous structure connecting the orifice of the inferior vena cava to the atrial septum.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

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.