Wolff-Parkinson-White syndrome with an unroofed coronary sinus without persistent left superior vena cava treated with catheter cryoablation

Coronary sinus anomalies are rare congenital defects which are usually coexistent with a persistent left superior vena cava and may be associated with cardiac arrhythmias. We report an unroofed coronary sinus without persistent left superior vena cava diagnosed during a catheter ablation procedure for Wolff-Parkinson-White syndrome. Diagnostic and therapeutic options and outcomes are discussed. This condition is of relevance to electrophysiologists performing catheter-based procedures, as well as cardiologists implanting coronary sinus pacing leads, who may encounter this anomaly in their practice.     

HEMIAZYGOS CONTINUATION TO CORONARY SINUS WITH NORMAL LEFT INNOMINATE VEIN

A case is described of absent hepatic segment of the inferior vena cava with hemiazygos continuation and drainage into the coronary sinus with associated atrial septal defect and patent ductus arteriosus. In all previously reported cases of inferior vena caval anomalies with persistent hemiazygos, the hemiazygos joined the homolateral superior vena cava. To our knowledge this is the first case to be reported of a patient who had hemiazygos continuation to the coronary sinus with a normal left innominate vein and a single right superior vena cava.     

Atresia of the right atrial ostium of the coronary sinus

A case of asymptomatic congenital occlusion of the ostium of the coronary sinus is described. The myocardial venous drainage was maintained via a persistent left superior vena cava as well via ectatic, widened atrial veins of the dorsal wall of the left atrium. The study shows that complete ostial occlusion of the coronary sinus does not reduce cardiac venous drainage. The view of the literature allows a comparison with the comprehensive classification of coronary sinus anomalies.     

Partial Unroofed Coronary Sinus Associated With Upper Septal Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

A 58 year old gentleman with complaints of palpitations and documented tachycardia was found to have a dilated right atrium, right ventricle and coronary sinus, which were due to partial unroofed coronary sinus without a left superior vena cava. He had upper septal ventricular tachycardia and atrio-ventricular nodal reentrant tachycardia, which was successfully treated by radiofrequency ablation.Key words: partial unroofed coronary sinus, dilated coronary sinus, tachycardia     

Left superior vena cava, a remnant of embryological development

A 46-year-old Brugada syndrome patient underwent insertion of a dual-chamber implantable cardioverter- defibrillator (ICD), revealing a left-sided superior vena cava (SVC), (figure 1), running, characteristically, left from the sternum and flowing into the great cardiac vein. Following this course, the atrial lead was placed in the right atrium (RA) (figure 2, arrow, note dorsal position). The ventricular lead was inserted through the connecting anonymous vein between left and right SVC (figure 1, double arrow), into the right SVC and right ventricle (RV). The presence of a left superior vena cava results from the persistence of the embryonic left anterior cardinal vein. This anomaly is present in approximately 0.5% of the general population and in 3 to 5% of persons with other congenital heart defects, as established by autopsy.     

A curious case of impossible coronary sinus cannulation

A 40-year-old male, diagnosed to have WPW syndrome and symptomatic with recurrent palpitations, was taken up for radiofrequency ablation. There was difficulty in coronary sinus cannulation. Coronary venogram revealed coronary sinus atresia with persistent left superior vena cava, and collateral venous pathways draining into the right atrium. This case is discussed for the rare coronary venous anomaly, its embryology and the difficulties in the management during electrophysiological studies.     

The usefulness of ventricular pacing during atrial fibrillation ablation in a persistent left superior vena cava: A case report

A 69-year-old woman with palpitations was referred to our hospital for a second session of atrial fibrillation (AF) catheter ablation. She had a history of AF ablation including pulmonary vein (PV) isolation and persistent left superior vena cava (PLSVC) isolation. Electrophysiologic studies showed the veno-atrial connections that had recovered. After PV isolation was performed, AF was induced by atrial premature contraction (APC) from the PLSVC, and AF storm occurred. During PLSVC isolation, AF was not induced by APC from the PLSVC. PLSVC isolation continued during sinus rhythm. The elimination of the PLSVC potential was difficult to confirm because of the far-field potential of the left ventricle. Then, we performed right ventricular pacing. The remaining PLSVC potential was identified. After that, the PLSVC isolation was successful during right ventricular pacing. Complications were not observed. The patient had no recurrence of AF thereafter.     

Tracer mixing: sites of tracer infusion and sampling

Controversy exists in the literature concerning the correct infusion and sampling sites in studies measuring substrate turnover rates. To investigate this problem, we examined the results obtained with various infusion and sampling sites in 7 anesthetized dogs. [1-14C]lactate was infused by a primed continuous infusion method in three different sites (the left ventricle, ascending aorta, and the aortic arch) in a sequential fashion; samples were obtained simultaneously from five sites (femoral artery, carotid artery, pulmonary artery, superior vena cava and inferior vena cava) for each of the three different infusion sites. [U-13C]lactate was also infused in a femoral vein and simultaneous samples were obtained in the carotid artery and femoral artery for analysis of the stable isotope. [14C]lactate analysis demonstrated that infusion of the tracer into the left ventricular chamber resulted in a uniform distribution in the systemic circulation. Infusion into the ascending aorta near the aortic valve resulted in uniform distribution of tracer in four out of five experiments. Tracer infusion into the aortic arch resulted in nonuniform systemic distribution of tracer. The [U-13C]lactate results showed that infusion into the femoral vein gives uniform systemic distribution, similar to that observed with left ventricular infusion. The pulmonary artery lactate specific activities varied from those in the superior vena cava. Thus, this study shows that the tracer must be infused in the left ventricle or upstream from this chamber to obtain optimal systemic distribution. Vena caval sampling, especially superior vena caval sampling, will not give a consistent mixed venous concentration of the lactate tracer. Therefore, aortic tracer infusion with vena caval sampling may lead to errors in determining substrate turnover values.     

Cardiac resynchronization therapy device implantation in a patient with persistent left superior vena cava without communicating innominate vein - should we proceed from the same side? - A dilemma revisited

Persistent left superior vena cava (PLSVC) is an uncommon congenital anomaly. We report a case of implantation of cardiac resynchronization therapy - pacemaker (CRT-P) device in a 38-year-old lady with idiopathic dilated cardiomyopathy. After left axillary vein puncture, we faced an unexpected entry of left subclavian to PLSVC draining into the coronary sinus (CS). The target posterolateral vein which had been identified before, seemed to have an acute angle at its entry into the CS. Hence, at this stage we were in a dilemma, whether to switch to the right side or to continue from the same side. We continued the procedure from the left side and completed it successfully after some manipulation and improvisation.     

Atrial natriuretic factor in the vena cava and sinus node

We investigated the localization of atrial natriuretic factor (ANF) mRNA and of immunoreactive ANF in the vena cava and sinus node of rat and, for comparative purposes, in atria and ventricles. In situ hybridization with an ANF cRNA probe revealed that the supradiaphragmatic portion of the inferior vena cava contains almost as much mRNA as the atria, whereas the levels were less in the superior vena cava and higher than in ventricles in the sinus node. Immunoreactive ANF (high Mr form) was found to be 22 times less abundant in the supradiaphragmatic vena cava and 148 times less abundant in the superior vena cava than in atrial cardiocytes. The wall of the supradiaphragmatic portion of the vena cava and the valve (eustachian valve) that separates the atrial cavity from that of the vein are made up of atrial-like cardiocytes containing secretory granules. The subendothelial area of the superior vena cava also contains atrial-like cardiocytes with secretory granules, whereas the outer portion of the vein is made up of "transitional cells" without or with only a few secretory granules. Secretory granules in the vena cava and nodal cells, as well as transitional cells, contain immunoreactive ANF. With immunocryoultramicrotomy, virtually all cells, whether atrial-like, transitional, or nodal, and even those without secretory granules, were found to contain immunoreactive ANF in their Golgi complex and in secretory vesicles in the vena cava and in the sinus node.     

Cryoablation for atrioventricular nodal re-entrant tachycardia associated with persistent left superior vena cava

Catheter ablation for atrioventricular nodal re-entrant tachycardia (AVNRT) in patients with persistent left superior vena cava (PLSVC) is challenging because of anatomical abnormalities of Koch's triangle associated with the enlarged coronary sinus ostium. We present the Case of successful ablation in a patient with PLSVC using the cryoablation technique. The ablation was successfully performed without damaging the conduction system by virtue of “cryomapping” and “cryoadhesion.” Cryoablation is a safe and efficacious alternative to radiofrequency catheter ablation for the treatment of AVNRT associated with PLSVC.     

A New Coronary Retroinfusion Technique in theRat Infarct Model: Transjugular Cardiac Vein Catheterization

Cell delivery via the retrograde coronary route boasts less vessel embolism, myocardial injury, and arrhythmogenicity when compared with those via antegrade coronary administration or myocardial injection. However, conventional insertion into the coronary sinus and consequent bleeding complication prevent its application in small animals. To overcome the complication of bleeding, we described a modified coronary retroinfusion technique via the jugular vein route in rats with myocardial infarction (MI). A flexible wire with a bent end was inserted into the left internal jugular vein and advanced slowly along the left superior vena cava. Under direct vision, the wire was run into the left cardiac vein by rotating the wire and changing the position of its tip. A fine tube was then advanced along the wire to the left cardiac vein. This modified technique showed less lethal hemorrhage than the conventional technique. Retroinfusion via transjugular catheter enabled efficient fluid or cell dissemination to the majority areas of the free wall of the left ventricle, covering the infarcted anterior wall. In conclusion, transjugular cardiac vein catheterization may make retrocoronary infusion a more safe and practical route for delivering cell, drug, and gene therapy into the infarcted myocardium of rats.     

Reconstruction of the Terminal of an Abandoned Fractured Unipolar Coronary Sinus Lead: a Feasible Solution to Restore Effective Cardiac Resynchronization Therapy

Complications related to coronary sinus lead are not infrequent in recipients of cardiac resynchronization devices. We describe the case of a patient with a biventricular implantable cardioverter defibrillator with persistent phrenic nerve stimulation, previous coronary sinus lead fracture, and severe left subclavian vein stenosis. The reimplantation of a new coronary sinus lead on the left side, ipsilateral to the original implant, was unsuccessful. In order to avoid more complex and risky procedures, we performed the repair of the fractured abandoned lead with the reconstruction of the unipolar lead terminal. Effective biventricular pacing was obtained with satisfactory electrical parameters and it was maintained at twelve months follow-up.     

Marshall to the rescue in cardiac resynchronization therapy: Left ventricular lead placement in coronary sinus ostial atresia

This case highlights the importance of proper identification of congenital anomalies of the coronary sinus for the successful placement of left ventricular lead during cardiac resynchronization therapy device implantation. We discuss an alternate route for left ventricular lead placement via the vein of Marshall when the coronary sinus ostium in the right atrium was atretic and was facing difficulty initially in detecting the anomaly.     

Isolation of persistent left superior vena cava during atrial fibrillation ablation

Persistent left superior vena cava is a rarely seen anomaly but it may be an arrhythmogenic source for paroxysmal atrial fibrillation. Furthermore, the complex anatomicregion between the left superior vena cava and the pulmonary veins may leads to misinterpretation of the pulmonary vein recordings during atrial fibrillation ablation. Approaches that might be helpful to overcome these problems are discussed in this case report.     

Video-Assisted Implantation of a Left Ventricular Lead and Intrathoracic Tunneling to a Right-Sided CRT-D Device

We describe two cases in which a biventricular implantable cardioverter defibrillator for cardiac resynchronization therapy had to be placed on the right side due to unsuitability of the left subclavian vein. Endocardial implantation of a left ventricular lead through the coronary sinus was previously attempted but was unsuccessful. Implantation of the epicardial left ventricular pacing lead was performed through video-assisted thoracic surgery on the left side. The connector end of the left ventricular pacing lead was tunnelized through the anterior mediastinum into the right pleural space. The right-sided pocket was then opened. A tunnel was created from the pocket to the thoracic wall, and the pleural space was entered over the second rib. The lead was retrieved from the right pleural space and connected with the Cardiac resynchronization therapy-device (CRT-D). Both procedures and postoperative periods were uneventful. Intrathoracic left-to-right tunneling of an epicardial left ventricular lead by video-assisted thoracic surgery is feasible and safe. It provides an alternative to subcutaneous tunneling.     

An unusual ‘bow tie’ image in pacemaker implantation

Upper venous system anatomic variations may cause difficulties during cardiac pacemaker implantation. Persistent left superior vena cava (PLSVC) and absent right superior vena cava could be an arrhythmogenic source of atrial arrhythmias and cardiac conduction disease. We represent dual-chamber pacemaker implantation in a patient with a very rare upper venous system anomaly, paroxysmal atrial fibrillation, sick sinus syndrome, that cause unusual fluoroscopic image.     

When the lead extractor becomes the assistant for new lead implant

With the increasing number of cardiovascular implantable electronic device upgrade and vein obstruction caused by previous leads, it is important to have alternative techniques to upgrade the device with the maintenance of functioning leads.We report an 83-year old male with 13-year old one-lead dual-chamber pacemaker, ischemic cardiac disease and pre-dialytic chronic kidney disease submitted to an upgrade to cardiac resynchronization therapy. A sub-occlusion in the transition of left brachiocephalic vein and the superior vena cava was documented. Re-permeabilization was only achieved with a TightRail? rotating dilator sheath over a guidewire with successful left ventricle lead implant.     

Hemodynamics mechanisms of changes in the right atrial pressure following intravenous injection of the depressor drugs

Changes of the right atrial pressure, superior and inferior vena cava flows, right ventricular myocardial contractility (first derivate of right ventricular pressure, dP/dt max) following i.v. injection of acetylcholine, histamine and isoproterenol, were studied in acute experiments on anaesthetized mongrel cats with artificial lung ventilation and opened chest. The right atrial pressure in those cases could be increased (I group of animals) or decreased (II group). In maximal shifts of right atrial pressure following acetylcholine injection, the superior vena cava flow increased but the inferior vena cava flow decreased in equal proportion. When the right ventricular myocardial contractility decreased more than the right atrial pressure was augmented, and when the cardiac negative inotropic effect was weak, the right atrial pressure was reduced. After histamine injection in both groups of animals, right ventricular myocardial contractility was increased on the same level, and changes of the inferior vena cava flow were insignificant. The right atrial pressure was elevated following greater increase of superior vena cava flow. Isoproterenol caused the positive cardiac inotropic effect and augmenting of the superior vena cava flow in both groups of animals. The right atrial pressure was elevated if the inferior vena cava flow increased and, on the other hand, when the inferior vena cava flow decreased the right atrial pressure was reduced. Thus different maximal changes of the right atrial pressure following i.v. injection of acetylcholine, histamine and isoproterenol could be explained by different hemodynamic mechanisms of the interaction between superior and inferior vena cava flow shifts and changes of the right ventricular myocardial contractility.     

Successful Implantation of Transvenous Pacing System via Persistent Left Superior Vena Cava and Coronary Sinus in Small Children

Transvenous pacemaker implantation tends to be difficult in the setting of a persistent left superior vena cava (SVC) and an absent or inaccessible right SVC. We report two small children in whom transvenous pacing leads were successfully inserted via a persistent left SVC. This technique was safe in our cases; however, favorable long-term result has yet to be demonstrated.