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.

Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate.

This report describes the case of a 13-day-old African American female with persistent hypoxia found to have an isolated right superior vena cava (RSVC) draining into the left atrium (LA) confirmed with contrast echocardiography.

Rare case of a superior sinus venosus atrial septal defect presenting with cyanosis.

Cyanosis in a newborn is commonly due to intracardiac shunts or duct-dependent lesions. Systemic venous anomalies are uncommon and sinus venous atrial septal defects do not usually present with cyanosis. We report the case of a 4-month-old female with persistent hypoxemia due to a right superior vena cava overriding a superior sinus venous atrial septal defect resulting in a right to left shunt.

Usefulness of a lead delivery system consisting of a fixed-shaped sheath and a lumenless bipolar lead in a patient with absent right and persistent left superior vena cava: A case report.

We report the case of an 84-year-old female with symptomatic bradycardia due to a complete atrioventricular block, who carried absent right and persistent left superior vena cava (SVC). Implantation of a pacing lead, particularly within the right ventricle (RV) in a patient with this venous anomaly is accompanied by technical difficulties. However, the apparatus consisting of a fixed-curve sheath (Model C315-S10, Medtronic, Inc., Minneapolis, MN, USA) and a lumenless fixed-screw pacing lead (Model 3830, Medtronic), allowed a rapid delivery into the RV without any complications. By rotating the Model C315-S10 sheath in the counterclockwise direction in the right atrium, its tip faced the tricuspid orifice, advanced across the tricuspid valve and confronted the RV lower septum near the apex. Then the RV-lead was fixed with acceptable pacing and sensing parameters. Utilizing a lumenless pacing lead and a preformed sheath to deliver it is a novel approach that could be helpful in pacemaker implantation in patients with absent right and persistent left SVC.

Pacemaker implantation in a patient with isolated persistent left superior vena cava using a delivery catheter: a case report.

Background: Persistent left superior vena cava (PLSVC) with absent right superior vena cava, also termed 'isolated PLSVC', is extremely rare. Permanent pacemaker implantation in patients with isolated PLSVC is often difficult by the usual subclavian approach due to the unique anatomy. With the advent of delivery catheters in recent years, implantation using the same system has been reported. Case summary: A 47-year-old woman with symptomatic sick sinus syndrome was admitted to our institution for permanent pacemaker implantation. Preprocedural cardiac multidetector computed tomography (MDCT) showed isolated PLSVC. We performed pacemaker implantation successfully via the left subclavian approach, using the C315 delivery catheter system. The leads were stable on chest radiography, and the sensing and capture thresholds were unchanged. After the procedure, we integrated the delivery catheter images with cardiac MDCT using Ziostation, and they were well matched with the fluoroscopic images. At the 1-month follow-up, the patient was free of heart failure symptoms and had decreased levels of N-terminal prohormone of brain natriuretic peptide. Discussion: The C315 delivery catheter system was considered an option for permanent pacemaker implantation in patients with isolated PLSVC. When performing permanent pacemaker implantation in patients with unusual venous anatomy, integrating the delivery catheter images with cardiac MDCT allows for appropriate preoperative catheter selection.

Unroofed coronary sinus, left-sided superior vena cava and mitral insufficiency: A case report and review of the literature.

BACKGROUND: Unroofed coronary sinus (UCS) is a rare subtype of atrial septal defect. It is frequently associated with a persistent left superior vena cava and is often part of a more intricate cardiac malformation. CASE SUMMARY: This report describes a rare case of an adolescent patient with UCS featuring atrial situs solitus, absence of the right superior vena cava and a persistent left superior vena cava draining into the left atrium consistent with total unroofing of the coronary sinus. This was associated with concurrent severe mitral insufficiency secondary to redundant and prolapsing leaflets, and a substantial left-to-right shunt across the coronary sinus orifice. A comprehensive examination of the existing literature is included, shedding light on the diagnostic challenges of UCS and describing the available surgical options within the context of mitral valve surgery. CONCLUSION: UCS is a complex condition requiring careful consideration of associated anomalies and a tailored surgical approach.

Prenatal diagnosis of persistent left and absent right superior vena cava.

We report a case of persistent left superior vena cava (LSVC) with absent right superior vena cava (RSVC) diagnosed prenatally. At 27 weeks' gestation, routine fetal ultrasonography showed an abnormal four-chamber view. Fetal echocardiography revealed a markedly enlarged coronary sinus in the four-chamber view. An absent RSVC and a persistent LSVC were demonstrated in the three-vessel view. No additional cardiac malformations were seen before birth. Postnatal imaging confirmed the prenatal diagnosis. The combination of persistent LSVC and absent RSVC without any other cardiac malformations is an extremely rare cardiac anomaly and less commonly detected in utero. Identification of a dilated coronary sinus in the four-chamber view, with atypical features in the three-vessel view, may lead to the antenatal diagnosis of this condition.

Partial anomalous systemic venous drainage, right superior vena cava to the left atrium in nonisomeric patient.

The most encountered anomalous systemic venous drainage is a persistent left superior vena cava that drains into the right atrium through the coronary sinus. A much rarer anomalous systemic venous drainage is that of isolated anomalous drainage of a normally positioned right superior vena cava (RSVC) into the left atrium (LA). This has been reported in approximately 20 patients, with the diagnosis usually made by cardiac catheterization. We report the youngest case diagnosed at the age of 3 h after birth with hypoxemia resulting from anomalous drainage of a normal RSVC into the LA. This was diagnosed noninvasively by echocardiography and confirmed by cardiac computed tomography angiography.

Absent right superior vena cava and persistent left superior vena cava: An incidental finding.

A patient with Marfan syndrome undergoing Bentall operation was found to have an absent right superior vena cava and persistent left superior vena cava. The dilation of coronary sinus raised the suspicion of persistent left superior vena cava. The diagnosis was confirmed by agitated saline contrast echocardiography and computed tomography of the chest.

Slittable sheath supported right ventricular pacing lead implantation in persistent left superior vena cava with absent right superior vena cava: a case report.

BACKGROUND: Persistent left superior vena cava (PLSVC) is the most common variant of systemic venous drainage. In the absence of the right superior vena cava (RSVC), implantation of a right ventricular pacing lead may be challenging. Therefore specific implantation techniques and experiences in PLSVC are worth reporting. CASE PRESENTATION: We present a case report of a 90-year-old Caucasian female patient with PLSVC during single chamber pacemaker implantation due to the third-degree atrioventricular block. With common implantation techniques, we did not even reach the right ventricle. Therefore slittable CPS Direct ™ Universal sheath was employed to overcome the acute angle from PLSVC to tricuspid valve and ensure more fixation stability for longer 100-cm right ventricular lead placement. CONCLUSION: This case demonstrates safe implantation of 100-cm long right ventricular bipolar active fixation pacing lead using common slittable CPS Direct ™ Universal sheath after failed attempts with "C" and "J" stylet shaped electrode. This sheath provides different angle towards tricuspid valve and more fixation stability in patient with PLSVC and absent connection to right atrium.

Right Superior Vena Cava Connected Left Atrium with Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum: An Unusual Cause of Paradoxical Embolism.

A six-month-old girl presented with history of convulsion and cyanosis, was eventually found to have a right superior vena cava connected to left atrium with anomalous upper right pulmonary vein return and without atrial septal defect. Diagnosis was confirmed by cardiac magnetic resonance imaging. The patient underwent a successful cardiac surgery correction, routing SVC to right atrium and repair of right upper pulmonary vein to left atrium.

An Ectopic Primary Pacemaker in Association with Isolated Persistent Left Superior Vena Cava.

We are reporting a case of a young Nepalese man, who was not known to have any past medical history, and who presented with palpitations. An electrocardiogram showed negative P-waves atrial rhythm in II, III, arteriovenous fistula, and V3-V6 with a variable block at 90-130 bpm. No positive "normal" P-waves were demonstrated in any tracing. He was found to have a congenital absence of the right superior vena cava (RSVC) along with persistent left superior vena cava (PLSVC) a condition also called isolated PLSVC (IPLSC). He underwent a treadmill stress test for further evaluation which showed a normal chronotropic response and the same persistent negative P-waves morphology. An invasive electroanatomical and activation mapping showed an absence of RSVC, the earliest atrial activation site in the anterior side of the inferior vena cava (IVC), and the absence of normal (positive) P-waves/normal sinus node (SN) activation. Considering all the available clinical data together, we believe that the patient was living with an ectopic pacemaker node that acts as a primary node and originated in the IVC/right atrium instead of the normal expected SN position. Given the high risk of complete sinoatrial nodal block in case of radiofrequency ablation, the patient was kept on medical treatment with a beta-blocker which was effective in controlling his symptoms and atrial arrhythmia.

Isolated Right Superior Vena Cava Drained to Left Atrium in a Child with Waardenburg Syndrome and Neurofibromatosis Type I.

Isolated right superior vena cava (SVC) draining to the left atrium (RSVC-LA) is an extremely rare cyanotic congenital heart disease (CHD). Such lesion is easily missed with improper scanning or inattentive interpretation of echocardiography. This can result in potential systemic severe complications. We report a child with RSVC-LA who has two significant non-cardiac co-morbidities, including Waardenburg syndrome and n neurofibromatosis type I (NF1). This patient was referred to cardiology assessment due to unexplained low saturation and was diagnosed as RSVC-LA; however, fortunately not yet showing complications of systemic thromboembolic phenomenon (STEP).

Permanent pacemaker implantation in a patient with persistent left superior vena cava with an absent right superior vena cava: A case report.

Persistent left superior vena cava (PLSVC) is a common congenital anomaly of the thoracic venous system. PLSVC affects 0.3-0.5% of the general population, with 0.09-0.13% having PLSVC with an absent right superior vena cava. A 92-year-old woman was transported to our hospital by ambulance because of syncopal attacks lasting a few seconds. Electrocardiography showed paroxysmal supraventricular tachycardia and sinus arrest for up to 10 s with syncope when the arrhythmia stopped. This occurred repeatedly, and a diagnosis of sick sinus syndrome was made. The patient was scheduled for dual-chamber permanent pacemaker implantation. She had isolated PLSVC, and pacing lead was inserted into the right atrium through the PLSVC from her left subclavian vein. The "J" pre-shaped stylet was used to introduce the lead via the tricuspid valve to the right ventricular outflow tract (RVOT) and placed in the septum of RVOT. Another lead was advanced into the right atrial appendage using the "J" pre-shaped stylet. After three years of implantation, fluoroscopy showed a stable position of pacing leads, and pacemaker interrogations showed no major changes in parameters. .

Pacemaker implantation using the SelectSecure system for a patient with persistent left superior vena cava and absent right superior vena cava: Insights into techniques for stable lead fixation.

The techniques for successful pacemaker implantation via the PLSVC with the SelectSecure system (Medtronic, Minneapolis, Minnesota, USA) are unknown. Regarding the techniques, we presented a case in which we implanted a pacemaker via the PLSVC in patient with absent RSVC using the SelectSecure system.

Pacemaker Implantation in Patient With Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava.

Persistent left superior vena cava (LSVC) is an asymptomatic congenital heart disease. It is usually found incidentally on imaging, during central line placements or while undergoing electrophysiological procedures. We present a case of a 91-year-old female who initially presented with seizures and was diagnosed with tachy-brady syndrome. She was planned to undergo dual-chamber permanent pacemaker placement. However, during the procedure, she was incidentally found to have an LSVC without a right superior vena cava. Due to challenging anatomy, her pacemaker was changed to a single-chamber atrial lead pacemaker. This case highlights the clinical implications of this unusual structural anomaly, technical difficulties that arise alongside and solutions on how to overcome these issues in the context of pacemaker implantation.

Persistent left superior vena cava with absent right superior vena cava in adults: CT and clinical findings.

PURPOSE: Persistent left superior vena cava (PLSVC) with absent right superior vena cava (ARSVC) is rare. We evaluated CT and clinical findings of adults with ARSVC. MATERIALS AND METHODS: Our study included 11 adults with ARSVC (mean age, 63 ± 17 years). We evaluated CT findings of the azygos vein system and coronary sinus (CS) using thin slice data of > 64 MDCT. Arrhythmia and congenial heart disease were assessed. We compared the CS diameters between the ARSVC group and the control group of 120 adults with normal vena cava (mean age, 60 ± 4 years). RESULTS: Adults with ARSVC had no azygos vein arch and the hemiazygos vein continued to PLSVCs via the superior intercostal vein. Eight adults had arrhythmia including conduction disturbance and one arrhythmia was associated with atrial septal defect. All adults with ARSVC had dilated CS larger than 11 mm. The mean diameter of the CS in the ARSVC group was 18.4 mm, and that of the control group was 6.8 mm. CS diameter in the ARSVC group was larger than that in the control group (p < 0.001). CONCLUSION: Adults with ARSVC had dilated CS and no azygos vein arch. Four adults had conduction disturbance.

Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava and Discrete Subaortic Stenosis Diagnosed in a Patient with Sick Sinus Syndrome: A Case Report and Brief Review of the Literature.

A persistent left superior vena cava (PLSVC) is the most frequent anomaly of the venous drainage system. While both a right and left superior vena cava (SVC) are usually present, a unique, left-sided SVC, also known as an isolated PLSVC, accounts for only 10-20% of cases. It is frequently associated with arrhythmias and other congenital cardiac anomalies. Though it is usually an asymptomatic condition, it may pose significant problems whenever central venous access is needed. We report a case of an isolated PLSVC that was diagnosed incidentally during pacemaker implantation for sinus node dysfunction. The venous anomaly was associated with subvalvular aortic stenosis determined by a subaortic membrane; this particular association of congenital cardiovascular anomalies is a rare finding, with only a few cases reported in the literature. We aim to highlight the clinical and practical implications of this condition, as well as to discuss the embryonic development and diagnostic methods of this congenital defect.