A rare case of isolated persistent left superior vena cava diagnosed by echocardiography.

BACKGROUND: The persistent left superior vena cava (PLSVC) is an infrequent vascular variant. PLSVC with absent right superior vena cava, also known as isolated PLSVC, is an exceptionally rare entity. In this case we present a patient with isolated PLSVC draining to coronary sinus, diagnosed incidentally during echocardiography. CASE PRESENTATION: A 35-year-old man underwent a transthoracic echocardiography which showed an enormously dilated coronary sinus. Hand-agitated saline was injected via peripheral intravenous cannulas. The contrast appeared firstly in the coronary sinus before it opacified the right atrium. Since this was also visible by the right antecubital saline injection, it indicated an extremely rare case of PLSVC with the absence of right superior vena cava which was confirmed by cardiac magnetic resonance. CONCLUSIONS: The finding of a distinctively dilated coronary sinus in echocardiography led us to further investigation using agitated saline that revealed an infrequent anomaly termed isolated PLSVC. The in-depth diagnosis of this vascular variant is crucial considering that it may lead to important clinical implications, such as difficulties with central venous access, especially in the current era of a rapid development of cardiac device therapies.

It's a long way to the top! Congenital venous anomalies and left isomerism limiting atrial fibrillation transcatheter ablation.

BACKGROUND: Atrial fibrillation is the most common sustained cardiac arrhythmia in adults and it is associated with a high burden of mortality and morbidity worldwide. Catheter ablation is increasingly used to improve symptoms and prognosis in selected patients. Lower limb venous access with subsequent transseptal approach to the left atrium is the standard procedure for atrial fibrillation catheter ablation. CASE PRESENTATION: We report an unusual case of complex venous anomaly with a left-sided inferior vena cava with hemiazygos continuation to a persistent left superior vena cava draining in an enlarged coronary sinus in a patient with persistent atrial fibrillation scheduled for transcatheter ablation. DISCUSSION: Lower limb venous anomalies may limit a standard transseptal approach to the left atrium thus precluding an effective catheter ablation procedure for atrial fibrillation. Alternative interventions, such as unconventional percutaneous access, thoracoscopic approach and "ablate and pace" procedures, may be necessary in patients with symptomatic atrial fibrillation and complex venous anomalies.

Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Absent Superior Vena Cava: An Uncommon Cause of Anomalous Systemic Venous Return.

We report a case of a one-year-old boy with tetralogy of Fallot, who was preoperatively diagnosed to have an associated systemic venous anomaly. Computed tomography confirmed the absent superior vena cava, and the case was managed with an appropriate cannulation strategy. Preoperative diagnosis and thorough planning of this rather benign anomaly were imperative for the successful outcome of this case. Clinical and surgical implications of this anomaly are discussed in this report.

Interrupted inferior vena cava draining into the coronary sinus associated with circumflex artery to coronary sinus fistula.

In an adult patient, coronary artery fistula involving the circumflex artery (CX) connected to the coronary sinus caused aneurysm of the left main and CX associated with ectasia of the intermediate branch. The patient had posterolateral infarction with severe ischemic mitral regurgitation and moderate tricuspid regurgitation. A rare venous return anomaly was also present. The inferior vena cava, which was interrupted at the level of the liver, continued with the hemiazygos to drain into a persistent left superior vena cava, which in turn drained into the coronary sinus. Surgery included CX closure and mitral and tricuspid repair. The strategy had to be adapted to the anatomy. The fistula was dissected and snared for cardioplegia delivery, and venous return was achieved by cannulation of the superior vena cava and femoral vein. The procedure was uneventful, and 4 years later the patient is asymptomatic.

Persistant Left Superior Vena Cava with and Without Right Superior Vena Cava: Significance of Prenatal Diagnosis.

This study aims to define the associated anomalies with PLSVC, and to compare single PLSVC and bilateral superior vena cava in terms of accompanying anomalies and pregnancy outcomes. This was a retrospective study of the fetuses diagnosed with single and/or bilateral SVC at a tertiary fetal medicine center during 8 years. We detected 16 cases of single PLSVC and 84 cases of bilateral SVC. We found an association between the PLSVC and cardiac and extracardiac anomalies. Comparison between single PLSVC and BSVC cases revealed significant differences in the occurrence of heterotaxy and right isomerism. The study highlights the importance of prenatal diagnosis in PLSVC cases. Isolated PLSVC with situs solitus may be considered a benign finding, but larger studies are needed to understand the clinical implications of PLSVC in relation to chromosomal anomalies. Routine screening protocols should include three-vessel and trachea views to detect PLSVC.

Left main coronary artery aneurysm with fistula to superior vena cava: A challenging case.

Coronary artery fistulas (CAFs) are rare congenital coronary artery abnormalities, with direct communication between a coronary artery and a cardiac chamber, great vessel or other structure. We report here, a rare case of a 25-year-old male with CAF from the aneurysmal left main coronary artery to the superior vena cava detected on echocardiography and computerized tomography (CT) coronary angiography.

Surgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report.

The normal anatomical course of right upper lobe pulmonary vein involves drainage anteriorly to the pulmonary artery, ultimately reaching the left atrium. However, anomalies can occur with the most common variation involving the convergence of the right upper lobe pulmonary vein with the superior vena cava. In a rare pulmonary vascular malformation, the anomalous right upper lobe pulmonary vein takes a path between the right pulmonary artery and right main bronchus [1]. During a clinical consultation, a patient presented in our hospital with this specific anomalous right upper lobe pulmonary vein, along with an atrial septal defect and a patent ductus arteriosus. As a consequence of this aberrant positioning, the right upper lobe pulmonary vein was compressed between the pulmonary artery and trachea, leading to pulmonary vein obstruction. Thus, a successful pulmonary vein replantation was performed to correct the congenital malformation.

Giant right atrial aneurysm associated with persistent left cranial vena cava in a cat.

A 10-month-old female spayed Scottish Fold was referred to cardiology for incidental radiographic cardiomegaly. Echocardiography was suspicious for a right atrial or right auricular aneurysm. The differential diagnosis also included peritoneal-pericardial diaphragmatic hernia, mass lesion (cyst, granuloma, or neoplasia), or cardiac malformation. A giant right atrial aneurysm associated with a persistent left cranial vena cava was subsequently confirmed with computed tomography.

Bilateral inferior venae cava combined with the persistent left superior vena cava and hemiazygos continuation of left inferior vena cava with drainage into right atrium: A case report.

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition.

Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.

Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.

Repair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Heart Transplant Using a Donor Heart With Only a Persistent Left Superior Vena Cava: A Case Report.

A persistent left superior vena cava (PLSVC) is a congenital anomaly wherein the left superior cardinal vein fails to regress. We describe the case of a successful orthotopic heart transplant using a donor heart with a PLSVC and congenital absence of a right superior vena cava (SVC) in a recipient with normal anatomy. After donor cardiectomy, the donor organ's PLSVC was ligated near the insertion site into the coronary sinus. The recipient underwent cardiectomy such that the native SVC was left with a long right atrial cuff. A modified bicaval technique was used to anastomose the recipient's right atrial cuff directly to the donor's right atrial appendage. This technique restored the recipient's normal anatomy, and we demonstrated that donor hearts with a PLSVC and absent right SVC might be used for transplant. Without other disqualifying abnormalities, surgeons should consider accepting these organs for life-saving transplant operations.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

Multiple accessory pathways coexisting with a persistent left superior vena cava: a case report.

BACKGROUND: Wolff-Parkinson-White syndrome is characterized by a short PR interval (delta-wave), long QRS complex, and the appearance of paroxysmal supraventricular tachycardia. Patients with Wolff-Parkinson-White syndrome usually have one accessory pathway, whereas cases with multiple accessory pathways are rare. Persistent left superior vena cava is a vascular anomaly in which the vein drains into the right atrium through the coronary sinus at the junction of the left internal jugular and subclavian veins due to abnormal development of the left cardinal vein. The simultaneous presence of multiple accessory pathways and persistent left superior vena cava has not been reported before. CASE PRESENTATION: A 56-year-old Japanese man with a 5-year history of palpitations was referred for radiofrequency catheter ablation due to increased frequency of tachycardia episodes in the previous 2 months. Persistent left superior vena cava was confirmed by transthoracic echocardiography and computed tomography. An electrophysiological study revealed that the accessory pathways were located in the left lateral wall, anterolateral wall, and posteroseptal region. They were completely ablated with radiofrequency energy application. CONCLUSIONS: We reported an extremely rare case of a patient with multiple accessory pathways and persistent left superior vena cava. Our case may suggest a potential embryological relationship between the multiple accessory pathways and persistent left superior vena cava.

Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism (P < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism (P = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.

Computed tomography and magnetic resonance imaging of congenital thoracic systemic venous anomalies.

We present the imaging findings of thoracic systemic venous anomalies diagnosed by computed tomography and magnetic resonance imaging. Persistent left superior vena cava is the commonest anomaly of the thoracic systemic veins encountered either incidentally as an isolated finding or associated with congenital heart disease. Inferior vena cava (IVC) interruption with azygos continuation is the second most common anomaly, which may also be isolated or be associated with left isomerism syndrome. The article will also discuss other rarer systemic venous anomalies including retroaortic brachiocephalic vein and IVC drainage into the left atrium. Finally, the impact of pre-procedure reporting of thoracic systemic venous anomalies on the choice of intervention and patient outcome will be addressed.