Left Superior Vena Cava Research Articles

Genetically Associated Hypertrophic Cardiomyopathy Combined with Persistent Left Superior Vena Cava

Background: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy. An implantable cardioverter defibrillator (ICD) is an efficient way of preventing sudden cardiac death in these patients.
 Aim: Diagnosis and treatment of genetically associated hypertrophic cardiomyopathy.
 Case Report: We present a 28-year patient with a history of tachycardia, dizziness, transient chest pains, and anamnestic information on episodes of short-term loss of consciousness and fatigue. She has a positive family history of HCM and her uncle died young from sudden cardiac death (SCD). The electrocardiogram showed hypertrophy, which was confirmed with echocardiography and MRI. Genetic testing confirms PRKAG2 gene mutation. Holter24-hour ECG monitoring showed domination of sinus bradycardia after which it was recommended implantation of ICD. On implantation, persistent left superior vena cava (PLSVC) was discovered and the implantation side was changed. A bipolar Implantable Cardioverter Defibrillator was implanted.
 Conclusion: When HCM is confirmed at a young age, genetically associated HCM should always be considered. Early recognition of hereditary hypertrophic cardiomyopathy can facilitate better disease management and follow-up even before symptoms appear.
 Keywords: hypertrophic cardiomyopathy, PRKAG2 gene mutation, PLSVC, SCD, ICD.

Persistent Left Superior Vena Cava Draining Directly Into the Left Atrium With Occluded Coronary Sinus

Combined persistent left superior vena cava entering the left atrium with a congenitally atretic coronary sinus is a rare imaging finding. In the absence of a significant right-to-left shunt, it is generally asymptomatic and can be an incidental discovery. Assessing the anatomy of the cardiac vasculature is crucial before transcutaneous cardiac procedures. (Level of Difficulty: Intermediate.)

An unusual Echo finding in a patient with TOF: unusual RAC sign

We report a 13 years old girl with cyanotic heart disease who presented to the OPD of a tertiary care facility for the first time. Her echocardiography showed features of tetralogy of fallot and dilated coronary sinus. An unusual structure was visualized below the aortic valve in PLAX and 4 chamber view. It also had systolic colour flow in it (Figures 1-4). Origin of the structure could not be traced so a cardiac CT was planned for delineation of the anatomy. DISCUSSION; Tetralogy of fallot is associated with multiple other abnormalities, one of which is abnormal coronary origins1. Our patient had normal coronary origins although the coronary arteries were markedly dilated and LAD had a brief retro-aortic course before emerging onto the anterior interventricular groove. RAC (retro-aortic coronary) sign is well known sign on echocardiography for retro-aortic course of one of the coronary arteries.2 A typical RAC sign is shown in figure 6. Since our patient did not have normal sized coronaries so typical RAC sign was not seen on echo. The typical location of the structure of interest and systolic colour flow demonstration in it raised the suspicion of it being a coronary artery. Dilated coronary sinus was due to Left persistant SVC which is also one of the associations of TOF. CONCLUSION; RAC sign is typical for retro-aortic coronary artery course. Slight variation in the appearance can occur due to variability in anatomic features of the coronary of interest. But still it is fairly reliable to predict the course of a coronary artery.

Isolated Persistent Left Superior Vena Cava with Continuation Into the Hemiazygos Vein and Left Renal Vein.

Isolated Persistent Left Superior Vena Cava with Continuation Into the Hemiazygos Vein and Left Renal Vein.

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.

A Rare Case of Two Superior Vena Cava with Two Azygos Veins in a Cadaver

In embryonic life developmental malformation of thoracic veins can lead to anomalies of the Superior vena cava and major thoracic veins like Azygos system. Double superior vena cava and double azygos vein are rare congenital anomaly with the incidence of 0.3%. These types of congenital anomalies may or may not be presented with clinical symptoms. This case was noted during the routine cadaveric dissection of thoracic region and heart for medical students in department of Anatomy, we found double superior vena cava (persistent left superior vena cava) along with double (paired) azygos vein in a female cadaver. Right superior vena was formed by the union of right subclavian and right internal jugular vein which receives the arch of right azygos before it opened into the right atrium. Left superior vena cava was formed by the union of left subclavian and left internal jugular vein and it also receives the arch of left azygos vein before it entered into the right atrium. In present case coronary sinus was separated from left persistent superior vena cava and it opened in the persistent left superior vena cava. There was no communication between both the superior vena cava. On right side Azygos vein was normal and open into right superior vena cava. On the left side hemiazygos was in continuation with the accessory azygos vein and it also forming the arch and opened into persistent left superior vena cava. There were no other congenital anomalies present in the heart. These types of congenital anomalies extremely very rare where the left superior vena cava opens into the right atrium directly and coronary sinus opens in to this left superior vena cava. Majority of such types of cases remain asymptomatic. Some cases may be detected accidently during diagnostic or surgical procedures like cardiac angiography, cardiac catheterization, by- pass and dialysis or during autopsy or institutional cadaveric dissection.

Persistent left superior vena cava in a patient with an implanted dual-chamber pacing system in atrial fibrillation with tachycardia-bradycardia syndrome

The relevance of the study is conditioned by the problem of implantation of an artificial cardiac pacemaker in atrial fibrillation in patients with tachy-brady syndrome according to the standard scheme related to the presence of a congenital anomaly, such as persistent left superior vena cava. The purpose of the study is to develop an operative method of implantation of a permanent two-chamber pacemaker in patients with tachy-brady syndrome with concomitant pathology of the persistent left superior vena cava. Research methods are the generally accepted clinical and instrumental examination of the patient, including taking anamnesis and a standard cardiological examination, electrocardiography, transthoracic echocardiography, plain radiography, angiocardiographic examination, and multispiral computed tomography, which, along with a general analysis, confirm the presence of tachy-brady syndrome with atrial fibrillation and congenital anomaly in the form of persistent left superior vena cava in patients. The study presents a developed model of surgical implantation of a permanent two-chamber pacemaker to stabilise the condition of patients with atrial fibrillation related to tachy-brady syndrome with concomitant persistent left superior vena cava; the standard implantation mechanism included the introduction of a radiopaque agent to clarify the anatomical structure of the vascular bed, further, its entry from the subclavian veins into the persistent left superior vena cava and into the cavity of the right atrium through the venous coronary sinus was detected, and then a gradual introduction of an endocardial right ventricular electrode was performed into the subclavian vein through the tricuspid valve along with its further positioning in the apex of the right ventricle; therefore, a permanent two-chamber pacemaker can be successfully installed, creating conditions for restoring sinus rhythm in this group of patients, which is of practical importance for the field of medicine (Tab. 3, Fig. 4, Ref. 20). Keywords: atrial fibrillation, persistent left superior vena cava, sick sinus syndrome, pacemaker implantation, cardiac surgery.

Leadless pacing in a patient with persistent left superior vena cava

Leadless pacing in a patient with persistent left superior vena cava

Complex Adult Congenital Heart Disease: A Rare Case of Dextrocardia with Double-outlet Right Ventricle

Abstract Double-outlet right ventricle (DORV) is a complex and rare congenital heart disease (CHD), which is usually symptomatic early in childhood and thus diagnosed early. Dextrocardia is another rare CHD that can occur either as an isolated cardiac abnormality or may be associated with other complex cardiac defects. However, the co-occurrence of DORV and dextrocardia is rare, especially in adult life. We present a 35-year-old Nigerian lady with situs inversus totalis and complex CHD, which included dextrocardia with multiple congenital intracardiac shunts, DORV occurring with L-malpositioning of the great vessels, atrial septal defect, and ventricular septal defect with partial anomalous pulmonary venous drainage, persistent left superior vena cava as well as severe pulmonary stenosis.

Prenatal diagnosis of a left superior vena cava draining into the left atrium and atrial septal defect with ventricular septal defect

Prenatal diagnosis of a left superior vena cava draining into the left atrium and atrial septal defect with ventricular septal defect

Persistence of the left superior vena cava as an incidental finding in left internal jugular vein cannulation.

Persistence of the left superior vena cava as an incidental finding in left internal jugular vein cannulation.

A case of Ellis-van Creveld syndrome in Palestine

Background: Ellis-van Creveld syndrome (EVC) causes chondral and ectodermal abnormalities. Although the precise prevalence is still unknown, the Amish group in the United States most frequently reports this uncommon sickness. Case presentation: The reported case was of a 2-year-old female patient presented with dysmorphic facial and digital features, polydactyly, dwarfism, inability to walk normally, and multiple cardiac abnormalities. On examination, the patient\'s growth parameters were below the 5th percentile, with a weight of 10 kg, height of 72 cm, and head circumference of 45 cm (10th percentile). The patient had sparse, thin hair with bi-temporal narrowing and frontal bossing. The patient was advised to undergo surgery, which included AV canal repair, atrial septal defect closure, VSD closure, Mitral and tricuspid valve cleft closure, and left SVC tunneling to the right atrium. One week after the operation, the patient developed sudden bilateral visual impairment, with no hemorrhage or space-occupying lesions. The patient was discharged on day 15 after surgery, and although stable, the visual impairment remained. Conclusion: This case is believed to be one of the first cases in Palestine, as this disease is very rare worldwide. The outcomes of the condition are thought to be well predicted by prenatal discoveries.

218 PERSISTENT LEFT SUPERIOR VENA CAVA IN AN ADULT PATIENT WITH AN IMPLANTED PACEMAKER: A CASE REPORT

Abstract Introduction Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly, although infrequent in the general population (prevalence estimates as 0.2-3%). It begins at the junction of the left subclavian and internal jugular veins, passes through the left side of the mediastinum adjacent to the aortic arch, and mostly drains into the right atrium via the coronary sinus (CS). For the majority of cases it is asymptomatic and it is mostly detected incidentally. More rarely it can be associated with other congenital abnormalities, such as aortic bicuspid valve, aortic coartation or atrial septal defects. Besides, in less than 10% of the cases the left superior vena cava drains in the left atrium, leading to a right-to-left shunt. Lastly, it can lead to arrhythmic problems. Case presentation A 85-year-old man was admitted to the emergency department for syncope. Previous medical history was unknown and there was no clinical documentation available. The electrocardiogram showed an atrial fibrillation and some pacemaker-induced beats. An echocardiogram was performed, which showed dilation and dysfunction of the right chambers and a dilated CS. Moreover, the same exam revealed the two pacemaker leads passing through the CS and ending their course into the right atrium and ventricle. The chest X-ray displayed the pacemaker leads passing to the left side of the aorta. Furthermore, a computed tomography angiography was performed in the suspicion of a pulmonary embolism. The exam showed small vascular filling defects in arteries of the right inferior lung lobe, but also the presence of two superior vena cavas, with the left one draining into the CS. The pacemaker leads passed from the left subclavian vein through the left superior vena cava reaching the right chambers. Therefore, the diagnosis of PLSVC was confirmed. Discussion and Conclusions Despite the benign natural history of this diagnosis, it is important to know the existence of this condition. PLSVC can be suspected when a dilated CS is detected on a transthoracic echocardiogram, with a parasternal long axis view. The best projections to visualize the dilatation of CS are also the apical four-chamber and the subcostal view. Besides, the direct detection of left superior vena cava can be obtained with a suprasternal view. The presence of the right superior vena cava should also be confirmed with a subcostal or suprasternal view. The differential diagnosis for a dilatation of the CS includes anomalous pulmonary venous return with a pulmonary vein draining in the CS, the “unroofed coronary sinus”, a tricuspid regurgitation with a jet directed towards the CS, elevated right-chamber filling pressures. The diagnosis of PLSVC can be confirmed with a saline contrast echocardiography (“bubble-test”) or with other radiological investigations (computed tomography or magnetic resonance). It should be reported in radiological reports even when it is an incidental finding because of its clinical relevance: it is essential to know its presence in advance in invasive procedures, such as pacemaker implantation, ablative procedures, cardiac surgery, because it could affect the proper approaches and could lead to complications. Moreover, in a minority of cases, it can be associated with other congenital abnormalities or with arrhythmic problems.

986 LEFT VENTRICULAR LEAD PLACEMENT FOR BIVENTRICULAR PACING INTO A LEFT LATERAL VEIN IN PATIENT WITH CONGENITAL ATRESIA OF THE CORONARY SINUS OSTIUM WHO UNDERWENT AORTIC VALVE REPLACEMENT

Abstract Introduction Coronary sinus ostial atresia (CSOA) is a rare congenital heart anomaly consisting in the occlusion of the coronary sinus (CS) outlet in the right atrium. It is one of the causes of placement left lead failure in CS during cardiac resynchronization therapy (CRT) device implantation. The persistence of the left superior vena cava is the most common pathway that allows venous blood flow to drain into the right atrium and can represent a new possible way to reach a left ventricular vein from the CS. Methods A 66-year-old male suffering from sarcoidosis was studied after an episode of syncope under exertion. EKG showed a 1st degree atrioventricular block and a left bundle branch block (LBBB) with wide QRS (170 ms). Echocardiography found left ventricle dilatation, severe systolic dysfunction, and a severe low-flow low-gradient aortic stenosis for which he underwent aortic valve replacement. After surgery and finding of 2nd degree type II atrioventricular block, CRT implant was performed but the procedure was complicated by the finding of CSOA. The insertion of the left quadripolar lead along a left branch of the occluded CS was achieved with a push-and-pull technique of the lead over a guidewire, after the retrieval and the cannulation of the persistent left superior vena cava. Results A new approach was applied on a rare case of CSOA found during CRT procedure. The contrast medium (CM) in the left subclavian vein revealed the existence of the left superior vena cava that was than cannulated. CM injection in CS highlighted a posterior left branch that was approached with a specific guidewire and a subselector after selective venography. With a fine technique of sliding of the lead on a tensioned guidewire, the quadripolar catheter was successfully placed in the left ventricular vein. Left-ventricle-right ventricle delays and catheter thresholds were tested and a final QSR duration of 90 msec in CRT mode was obtained. Conclusions This case-report shows a complex technique, reported in parallel with other similar procedures, to help the approach toward a rare anatomical variant of CSOA accompanied by persistent left superior vena cava when a CRT implant is needed after cardiac valve surgery in a patient suffering from sarcoidosis.

540 RAGHIB SYNDROME WITH VERY LATE CLINICAL PRESENTATION.

Abstract A 72-year-old woman who had been diagnosed with pulmonary emphysema and permanent atrial fibrillation was admitted to our emergency department with signs and symptoms of heart failure (NYHA class III). Transthoracic echocardiography (TTE) revealed dilatation of both right and left atria, dilatation and systolic dysfunction of the right ventricle (RV) and severe pulmonary hypertension. Combined severe mitral and tricuspid regurgitation was also noted and, after clinical stabilization, transesophageal echocardiography (TEE) was performed to better assess valvular anatomy and function. TEE revealed clear evidence of a pre-tricuspid left-to-right shunt with signs of significant RV volume overload, but with difficulties in defining the characteristics of a suspected atrial septal defect (ASD). CMR confirmed the presence of a large coronary sinus ASD (Panel A) as responsible for a significant left-to-right shunt (Qp/Qs ∼ 3,5, measured by phase-contrast imaging after correcting for the combined mitral and tricuspid regurgitation; Panel B), as well as severe RV dilatation and dysfunction. Pulmonary venous drainage was normal. Persistent left superior vena cava (SVC; Panel C) draining directly into the left atrium (LA) and a completely unroofed coronary sinus were unveiled. The extremely rare combination of an unroofed coronary sinus with abnormal systemic venous drainage from double SVC circulation (usually as a persistent left SVC) is also known as Raghib syndrome (or Raghib complex). In the ASD with completely unroofed coronary sinus there is no common wall separating the coronary sinus from the LA, making the depiction of the shunt anatomy particularly difficult to assess with echocardiography. When unrecognized in the perinatal age, clinical natural course of Raghib syndrome can vary based on the severity of RV volume overload, and late (or very late) clinical presentations have been rarely reported. CMR, with its large field-of-view capability and multiparametric approach, provides accurate definition of cardiac and great vessels anatomy and function allowing the full non-invasive depiction of most congenital abnormalities. In many cases, multimodality imaging is the key to reach a full depiction of these complex conditions, particularly when adult patients with possible comorbidities need to be evaluated.

105 A CASE OF RARE ATYPICAL ATRIAL STRUCTURE

Abstract A 27-year-old woman, without previous cardiological history, was admitted at our emergency department in the clinical context of an acute pericarditis. Echocardiography documented mild pericardial effusion (8 mm) without any sign of hemodynamic impact. The patient was then admitted at our Cardiology Unit. During the hospitalization a complete transthoracic echocardiogram showed a normal biventricular morphology and function, normal valvular function and found an anechoic structure on the postero-lateral left atrial wall (1.75×1.5 cm). To better characterize this accidental finding, a transesophageal echocardiography was performed unmasking a dilated coronary sinus with anomalous course. A subsequent cardiac computed tomography was performed showing anomalous systemic venous return with the persistency of the left superior vena cava draining into the coronary sinus. No other congenital anomalies were found. PLSVC is present in approximatively 0.5% of individuals in the general population and is the most frequent congenital malformation of the thoracic venous return. In about 90% coexists with the right superior vena cava and co-occur in almost 40% of patients with other congenital heart abnormalities such as atrial septal defect, aortic coarctation, bicuspid aortic valve, tetralogy of Fallot, transposition of the great vessels, anomalous connections of the pulmonary veins, dextroversion and cor triatatum. Patients usually are asymptomatic. Nevertheless, this congenital malformation may cause chest discomfort, cardiac arrhythmias, thromboembolic events, decreased exercise tolerance and syncope. Moreover, the correct identification of this condition has important clinical implications. In fact, it is a relative contraindication to the administration of retrograde cardioplegia during cardiac surgery. Furthermore, the placement of central venous-access line, pulmonary artery catheterization or the implantation of pacemaker/resynchronization leads could be difficult or can result in incorrect positioning. In these cases, it is recommended to access to the right heart through the right subclavian vein instead the left one. Legend: *: Anomalous course of coronary sinus; LA: Left Atrium; LV: Left Ventricle; Ao: Aorta; RA: Right Atrium; R-SVC: Right superior vena cava; L-SVC: Left superior vena cava; LSPV: Left superior pulmonary vein.

Double superior vena cava and left brachiocephalic vein agenesis: a rare systemic vein anomaly and potential source of cardiac implantable electronic device and central venous catheter placement complications.

Abnormal systemic vein development produces anomalous veins, which - in the case of persistent left superior vena cava and/or left brachiocephalic vein - exhibit considerable topographic and morphometric differences in comparison with their usual anatomy. The nature and extent of those developmental anomalies - detected during intravenous procedures, such as cardiac implantable electronic device (CIED) lead insertion or central venous catheter placement - may hinder the procedure itself and/or adversely affect its outcome, both at the stage of cardiac lead advancement through an abnormally shaped vessel and lead positioning within the heart. This may lead to problems in achieving optimal sensing and pacing parameters and in ensuring that the patient cannot feel the pacing impulses. These events accompanied a de novo CIED implantation procedure in the patient with a double superior vena cava and left brachiocephalic vein agenesis, who ultimately required reoperation.

Phenotypic findings and pregnancy outcomes of fetal rare autosomal aneuploidies detected using chromosomal microarray analysis

BackgroundAneuploidies are the most common chromosomal abnormality and the main genetic cause of adverse pregnancy outcomes. Since numerous studies have focused on common trisomies, relatively little is known about the association between phenotypic findings and rare autosomal aneuploidies (RAAs). We conducted a retrospective study of 48,904 cases for chromosomal microarray analysis in a large tertiary referral center and reported the overall frequencies, clinical manifestations, and outcomes of prenatal RAAs.ResultsA total of 90 RAAs were detected, of which 83 cases were mosaic trisomies and 7 were non-mosaic trisomies. Chromosomes 16, 22, and 9 were identified as the major chromosomes involving RAAs. The four predominant indications for prenatal diagnosis in our RAA cases were RAA-positive in noninvasive prenatal screening, advanced maternal age, ultrasound abnormalities, and high-risk for serum prenatal screening. Cardiovascular defects were the most frequently observed structural abnormalities, followed by musculoskeletal anomalies. Increased nuchal translucency and persistent left superior vena cava, the major soft marker abnormalities involved, were also observed in our RAA cases. Clinical outcomes were available for all RAAs, with 63 induced abortions and 27 live births recorded.ConclusionsVariable phenotypes and outcomes were observed, which were highly heterogeneous in cases of prenatal RAAs. Thus, a cautious and comprehensive strategy should be implemented during prenatal counseling for RAAs.

MODIFIED DEEP TRANSGASTRIC TRANSESOPHAGEAL ECHOCARDIOGRAPHY IMAGING OF SUPERIOR VENA CAVA

<h3>Background</h3> Superior vena cava is usually interrogated intraoperatively by a standard bi-caval view as suggested by American Society of Echocardiography. A modified deep transgastric transesophageal echocardiography view is suggested that will superior interrogation of the superior vena cava intraoperatively. <h3>Objective</h3> Evaluation of a modified deep transgastric imaging of superior vena cava in patients undergoing cardiac surgery. <h3>Methods and material</h3> A modified deep transgastric imaging of superior vena cava was performed in children undergoing cardiac surgery. The deep transgastric interrogation with color Doppler blood flow map showed the venous return pattern. A pulse wave Doppler signal analysis was also feasible. The position of the tip of a central venous catheter tip that was inserted through the internal jugular vein could be seen. An agitated saline contrast injected into the left upper limb could exclude the presence of a left superior vena cava. In patients undergoing a undergoing a bi-directional Glenn shunt, considerable length of superior vena cava was visualized. In a child with a partial anomalous pulmonary venous drainage and a superior sinus venosus ASD, a point of the pulmonary vein joining the superior vena cava could be identified as it was possible to interrogate a considerable length of superior vena cava compared to the standard mid esophageal bi-caval view. The deep transgastric TEE interrogation of superior vena cava revealed a residual gradient in the superior vena cava following a sinus venosus atrial septal defect repair that was corrected at the same sitting. A color Doppler blood flow map across the superior vena cava following removal of a directly inserted venous cannula showed turbulence and a continuous-wave Doppler signal analysis revealed a residual gradient that was immediately corrected by the surgeon in another patient. <h3>Conclusion</h3> In conclusion, the deep transgastric TEE view of the superior vena cava is clinically useful. The doppler alignment is parallel to SVC flow (less than 20 degree) which is superior to bi caval view, where the doppler alignment is almost perpendicular to the flow. The probable difficulties that may be encountered that would interfere with proper visualization are: (i) poor contact of the probe with cephalad aspect of the stomach, (ii) air in the stomach, (iii) use of inadequate contact jelly, and (iv) lack of experience. There is a deep learning curve that can be a practical impediment but once honed, it could be a really useful adjunct to our routine trans-esophageal views.

Incidental Vascular Findings in Computed Tomography Performed in the Qualification for the TAVI Procedure.

Transcatheter aortic valve implantation (TAVI) or transcatheter aortic valve replacement (TAVR) is now a very widespread treatment method for symptomatic and severe aortic stenosis as an alternative for patients at intermediate or high risk of surgery or contraindications to surgery. The key role of imaging examinations before TAVI is to assess the morphology of the aortic valve, the routes of surgical access, and non-cardiac and extravascular structures. The objective of this article is to present and discuss the importance of selected accidental vascular findings in computed tomography examinations of the heart and large vessels performed in the TAVI qualification procedure: persistent left superior vena cava (SVC) with absent right SVC, right aortic arch, ectopic right coronary artery ostium, and left superior pulmonary vein draining into left brachiocephalic vein.