Systemic Venous Anomalies Research Articles

Persistent Levoatriocardinal Vein in Association with Complete Duplication of SVC and IVC

AbstractPersistent levoatriocardinal vein (LACV) is a rare pulmonary venous anomaly characterized by persistence of embryonic pulmonary to systemic communication. It is commonly associated with obstructive lesions of the left heart, such as hypoplastic left heart syndrome and mitral atresia. Complete duplication of the superior vena cava (SVC) and inferior vena cava (IVC) is rare, and occurs due to aberrations in the multistep development of the cardinal venous system. Coexistence of double SVC and IVC has been reported in the literature; however, their occurrence with persistent levoatriocardinal vein is hitherto unreported. The current case describes a rare coexistence of these complex pulmonary and systemic venous anomalies: persistent levoatriocardinal vein in a child with double SVC and IVC. The embryological development and possible aberrations leading to these anomalies are also detailed.

Recognizing Total Anomalous Pulmonary Venous Drainage (TAPVD) from Natural History to the Supportive Imaging Modalities

Congenital Heart Disease (CHD) prevalence was 8 until 9 for each thousand live births, and among all of the CHD, there is in one of condition that involved the malformation of the venous system that called Total Anomalous Pulmonary Venous Drainage (TAPVD). TAPVD can be found and to be diagnosed in all ages, not only in the childhood era. Adult individuals with TAPVD are susceptible to being misdiagnosed as having a significant secundum atrial septal defect if the condition is not known or suspected. A systematic history taking, physical examination, and another supportive imaging modalities is needed to ensure this structural anomaly. Keyword: Congenital Heart Disease, TAPVD, Venous System Anomalies.

Foramen ovale flap aneurysm in fetuses associated with and without heart defects: Prenatal diagnosis, imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes.

Isolated redundant foramen ovale flap aneurysm (RFOA) in the absence of restrictive foramen ovale is believed to be a cause for pseudocoarctation of aorta since the impediment of blood flow to the left heart can be severe, resembling the picture of left ventricular hypoplasia with retrograde aortic flow. The primary objective of the study is to find whether RFOA is always a benign lesion. The main focus of the study is to share my experience in particular on fetuses having redundant foramen ovale flap aneurysm developing into coarctation of aorta and to study the associated factors. Retrospective study (January 2020 to June 2023). All fetuses with RFOA associated with and without congenital heart defects were included. Fetuses with restrictive foramen ovale and RFOA with single ventricle hearts were excluded. The imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes with at least 3 months follow-up were presented. During the study period, a total of 1499 fetal echocardiography were performed. Twenty-two fetuses with RFOA were included. Fourteen fetuses had isolated RFOA and eight had associated abnormalities [extracardiac (n=5); intracardiac (n=2), Both (n=1)]. Genetic evaluation were performed only in fetuses with associated defects were normal. Postnatally all isolated RFOA fetuses had no aortic arch obstruction. Fetuses with associated aberrant right subclavian artery, isolated left superior vena cava, absent ductus venosus and ventricular septal defects developed aortic arch obstruction after birth. RFOA causes smallish left ventricle in fetuses with tetralogy of Fallot which recovered to normal size postnatally. Isolated RFOA can be benign, however, if it is associated with cardiac or extracardiac anomalies predominantly resulted in aortic arch obstruction. Though it is a cause for pseudocoarctation of aorta, through postnatal reassessment of aortic arch is mandatory. Careful search for intracardiac and systemic venous anomalies is recommended. It created confusion regarding adequacy of left ventricle when associated with congenital heart defects.

Collaterals in congenital heart disease: when and how to treat?

The development of collateral circulation is not that rare in patients with congenital heart defects. These collaterals can affect cardiovascular hemodynamics and cause systemic arterial desaturation, which arises the question whether these should be closed. To date, few if any reports have been published on the therapeutic management of collaterals in adult patients with congenital heart disease in heart failure (HF). The focus of this article is to provide a pragmatic approach in the assessment of collateral circulation of the patient with HF. By considering the underlying hemodynamics and overall effects of the collateral circulation, we aim to provide a practical tool useful in clinical decision making. The paper highlights mainly the systemic venous to systemic venous collaterals, systemic venous to pulmonary venous (or pulmonary venous atrium) collaterals, and pulmonary arterio-venous malformations. Systemic venous anomalies are frequent and reported in 20% to 40% of patients who underwent Glenn or Fontan procedure. A reduction in effective pulmonary blood flow, coupled with increasing oxygen demands with growth, as well as a pressure difference between the higher pressure caval venous system and lower pressure atria (so called decompressing collaterals) are potential causes of collateral formation. Whether angiogenesis de novo or reappearance of embryological venous channels is responsible for collateral formation remains to be elucidated.

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.

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.

Clinical Case Reports 1971-1974

In this paper five case reports were presented and include congenital heart disease in de Lange Syndrome, management of growth retardation in steroid dependent asthma, systemic venous anomalies and partial heterotaxia with normal heart, Ebstein’s malformation of the tricuspid valve with atresia, and superior vena caval obstruction in total anomalous pulmonary venous connection. For each case report, clinical, chest x-ray, electrocardiogram and other pertinent findings were presented. This was followed by discussion of etiology, diagnosis, and treatment options, as appropriate.

Clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects: A retrospective study from two centres in Southern India.

Recent advances in fetal ultrasound imaging, especially four-dimensional (4D) spatio-temporal image correlation techniques permit detailed evaluation of the fetal venous system and its abnormalities. In this report, we present the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in southern India. To report the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in Southern India. Retrospective study (October 2017 to March 2022). All fetuses referred with abnormal cardiac imaging findings who were diagnosed to have anomalies of systemic venous return in the absence of significant intracardiac defects were included. Imaging techniques like 2D with color and 4D spatio-temporal image correlation fetal imaging were used to evaluate systemic venous anomalies. Systemic venous anomalies were grouped into cardinal venous anomalies and umbilico-portosystemic venous anomalies. Thirty-nine fetuses were included; this represented 4.4% of all cardiac anomalies diagnosed during the study period. Cardinal venous anomalies were seen in 29 fetuses (74%); the mean gestation age at diagnosis was 25.5±3.4weeks. Absent dilated coronary sinus was associated with anomalous drainage of superior caval vein through completely unroofed coronary sinus into the left atrium (n=3) which resulted in cyanosis after birth in all infants. Among the three cases, one underwent surgery at 6 months of age due to a progressive increase in cyanosis (Spo2 78%) and others are awaiting surgery. A prenatal genetic evaluation was performed in 17 (58.6%). Cardinal venous anomalies were not associated with genetic abnormalities. Live births occurred in all fetuses with cardinal venous anomalies. Umbilico-portosystemic venous anomalies were seen in 10 (26%). The mean gestation age at diagnosis was 26.5±4.5weeks. Except in a fetus with the extrahepatic portosystemic shunt (umbilical vein to iliac vein), the genetic evaluation was normal in our cohort. Extracardiac anomalies (n=3/10; 30%) were seen in both intra (n=1) and extrahepatic portosystemic shunts (n=2). Anomalies of systemic venous return in the absence of significant intracardiac, extracardiac, and genetic abnormalities carried an overall favorable prognosis in our cohort. Precise characterization of anatomic details using advanced imaging techniques helps in the understanding of this complex three-dimensional anatomy and in the prognostication of these anomalies.

Abnormal drainage of inferior vena cava to left atrium combined with atrial septal defect: A case report

Systemic venous anomaly is less common clinically, and ectopic connection of inferior vena cava is less common clinically than ectopic connection of superior vena cava. When the inferior vena cava is abnormally connected to the left atrium, a low atrial septal defect can be combined. It is difficult to make preoperative diagnosis of this kind of disease clinically. Blind occlusion of the ASD (atrial septal defect) will cause the iatrogenic inferior vena cava flowing back to the left atrium completely or partially. Conventional median thoracotomy for repair of such an atrial septal defect should be performed with more caution. It has been reported that after repair of an atrial septal defect, the inferior vena cava was mistakenly separated into the left atrium. Therefore, we suggest that preoperative evaluation of inferior atrial septal defect should be with more cautious. When transthoracic echocardiography (TTE) cannot fully explain the clinical symptoms, Ultrasound contrast may be considered. During the repair of the inferior vena cava ASD, it is not only necessary to find the location of Euclidean valve, but also to accurately explore the inferior vena cava opening so as to avoid the iatrogenic inferior vena cava being separated into the left atrium.

Fontan procedure on deep hypothermic circulatory arrest: Short-term results and technique.

Various operative strategies are described for the Fontan procedure. In this study, we describe our short-term results and technique of Fontan procedure on cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). This was a retrospective study of 32 patients, median age of 6 years (4-19 years) and median weight of 20 kg (13-51 kg), who underwent Fontan procedure on CPB and DHCA from July 2016 to July 2021. The median CPB time was 125 min (77-186 min), the median DHCA time was 42 min (27-50 min), and the median Fontan pressure was 14 mmHg (10-18 mmHg). The median time to extubation was 4 h (1-20 h), the duration of chest tube drainage was 8 days (5-24 days), and the median intensive care unit stay was 4 days (3-8 days). The presence of heterotaxy was associated with longer duration of pleural drainage (P = 0.01). There was no operative mortality and no major adverse events such as seizures, gross neurological deficits, or arrhythmias in the postoperative period. Fontan procedure can be safely performed on CPB and DHCA with good operative results. This operative strategy may be used in special circumstances like in patients with situs and systemic venous anomalies and those requiring repair of a complex intracardiac defect. Long-term follow-up will be required to evaluate if this strategy has any impact on the neurodevelopmental outcome and the long-term sequelae of Fontan.

Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies?

BackgroundThe term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning.ResultsA total of 50 patients diagnosed by echocardiography with DORV referred from our institute between September 2018 and August 2020 were enrolled. The location of the ventricular septal defect (VSD) to the great vessels and the alignment of the great vessels to each other as well as other associated malformations that affect the surgical planning were assessed by MDCT. The patients were retrospectively classified into subgroups according to the relative positions of the great arteries, the relationship between the great arteries and the VSD, and the presence of associated malformations according to the classification of Society of Thoracic Surgeons database. The diagnostic value of MDCT and transthoracic echocardiography (TTE) in evaluation of associated cardiac malformations apart from double outlet right ventricle was compared in 20 patients, whom surgeries were performed and surgical results were obtained. Fallot type of DORV was the most common clinical type which represented 48% of the patients in which the VSD is committed to the aorta. The second most common clinical type was Taussig–Bing anomaly which represented 30% of the patients and the VSD was seen committed to the pulmonary artery. The atrio-ventricular septal defect (AVSD)/uni-ventricular type represented 16% of the patients in the current study, with pulmonary and systemic venous anomalies, heterotaxy syndrome and situs anomalies were commonly associated. A total of 53 malformations apart from DORV were found in 20 patients whom performed surgical interventions. MDCT detected different vascular and situs anomalies in those patients with 100% sensitivity and 100% positive predictive value compared to 71.4% and 94% respectively in TTE. On the other hand, in our study TTE was superior to MDCT for detection of intracardiac anomalies with 100% sensitivity and 100% accuracy compared to 87.5% and 95% respectively in MDCT.ConclusionsOur study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV. It is superior to echocardiography in providing a comprehensive mapping of the anatomy of complex anomalies especially that of extra-cardiac structures such as aorta, systemic veins, pulmonary veins, and pulmonary arteries. However, it can miss tiny intracardiac malformations. So combining the results of MDCT and echocardiography would be beneficial to guarantee the accuracy of diagnosis of DORV.

Abstract 10569: Risk Factors for Adverse Outcome in Prenatally Diagnosed Heterotaxy Syndrome in the Current Era

Introduction: Fetuses with heterotaxy syndrome (HS) and congenital heart disease continue to have worse outcomes compared to other lesions. We sought to explore differences between asplenia and polysplenia subtypes and to identify risk factors for poor outcome in the current era. Methods: All cases of fetal HS seen at our center between 1/2005 and 3/2018 were included in this study. Prenatal echocardiographic parameters and clinical outcomes were recorded. Findings were compared between the asplenia and polysplenia subtypes via Chi-square or Fisher’s exact analysis. Univariate logistic regression was utilized to identify prenatal variables predictive of fetal demise. Postnatal survival was examined with Kaplan-Meier curves and univariate Cox proportional hazards models. Results: The cohort consisted of 155 subjects: 86 with asplenia and 69 with polysplenia. There were 36 with biventricular (2V) and 119 with univentricular (1V) physiology. Asplenia subjects were more likely to have 1V physiology, common atrioventricular canal (CAVC), conotruncal anomaly, and pulmonary venous obstruction (all p<0.001). Polysplenia subjects were more likely to have systemic venous anomalies (p<0.001), complete heart block (CHB, p<0.001), ventricular dysfunction (p=.005), and hydrops (p=0.03). There were 105 subjects born alive, 7 fetal demises, 40 terminations, and 3 lost to follow-up prior to birth. Postnatal follow-up data were available for 96/105 (91%) subjects with median follow-up of 5.5 years (IQR 1.2-10.5). Estimated 5-year survival was 72% (95% CI 62-80) for the entire cohort and there was no difference in overall postnatal survival between polysplenia and asplenia subtypes (p=0.24). CHB and ventricular dysfunction were significant risk factors for both fetal demise and postnatal death. Pulmonary venous obstruction and CAVC were additional risk factors for postnatal death. Conclusions: Fetuses with HS continue to have high rates of postnatal mortality, particularly if CHB, ventricular dysfunction, or pulmonary venous obstruction are identified. CHB and ventricular dysfunction are also significant risk factors for fetal demise. Further research is needed to improve management of CHB and pulmonary venous obstruction in this high-risk group.

Systemic Venous Reconstructions During Pediatric Heart Transplantation.

Congenital heart disease continues to be an important indication for pediatric heart transplantation (HT) and is often complicated by systemic venous anomalies. The need for reconstruction, surgical technique used, as well as the outcomes of these have limited documentation. Descriptive, retrospective study of patients transplanted at Emory University between 2006 and 2017. We reviewed surgical data, follow-up, and interventions for patients necessitating venous reconstruction during transplantation. A total of 179 transplants were performed during the time period of which 74 (41%) required systemic venous reconstruction. Mean age at transplant was 6.3 (±6.16) years, and 74.3% of these patients carried a diagnosis of single ventricle; 51 (68.9%) of 74 patients required pulmonary artery reconstruction at the time of HT. Forty patients required superior vena caval reconstruction, while 22 patients required inferior vena caval reconstruction due to prior palliation or anomaly. Venous anomalies along with other anatomic features necessitated biatrial transplantation in four patients. Posttransplant evaluation revealed systemic venous stenosis in 14 (18.9%) of 74 patients. Eight (10.8%) patients required 12 interventions for the systemic veins. Patients with bilateral Glenn anastomosis prior to transplant were at high risk for the development of stenosis and needing interventions. Systemic venous complications were uncommon in those with native systemic veins without Glenn or Fontan procedure. Systemic venous reconstruction needs are high in pediatric HT. Posttransplant stenosis and the need for interventions are relatively common. Current techniques for systemic venous reconstruction for complex congenital heart disease patients may deserve further review to optimize these outcomes.

Echo Rounds: Partial Anomalous Systemic and Pulmonary Venous Return.

Anomalous drainage of a right-sided superior vena cava to the left atrium is a rare variant of systemic venous return anomalies. During the course of investigation for a heart murmur, an asymptomatic child was found to have a superior sinus venosus atrial septal defect with partial anomalous systemic and pulmonary venous drainage. Despite the right superior vena cava overriding the upper end of the atrial septum and draining into the left atrium, the child did not exhibit hypoxemia. Saline contrast transesophageal echocardiography suggested an explanation for the absence of hypoxemia.

Transcatheter closure of Abernethy Malformation associated with interrupted inferior caval vein and other systemic venous anomalies - ERRATUM.

Abernethy malformation is a rare entity. We report a 5-year-old boy presenting with severe pulmonary hypertension in whom Abernethy malformation and inferior caval vein interruption were diagnosed by CT angiography. In addition, the iliac veins were thrombosed with multiple venous collateral drainage. This abnormal venous anatomy caused difficulty in device closure of the Abernethy malformation, which was successfully closed using a vascular plug.

Persistent right umbilical vein: its incidence and clinical importance

Objective To investigate the incidence and concomitant findings of persistent right umbilical vein (PRUV). Methods The study was conducted by retrospective review of the data of 1856 patients who admitted to the Clinic of Obstetrics and Gynecology of Fırat University between December 2018 and December 2019 for the gestational examination between 14 and 28 weeks of gestation. The obstetric characteristics of the patients such as age, number of pregnancy, abortion, parity and body mass index were recorded. The diagnosis of PRUV was established in the conditions where portal vein reaches to stomach abnormally (roughly, course towards stomach instead of parallel course), fetal gall bladder locates in the medial of umbilical vein or umbilical vein connects to right portal vein abnormally instead of left portal vein. In the cases diagnosed with PRUV, the isolated persistent right umbilical vein or its association with other anomalies was recorded. Ultrasonography findings (minor markers) were also recorded in these cases. Results During the study period, 1856 pregnant women were evaluated for gestational examination. Five cases were established with the diagnosis of PRUV. Accordingly, PRUV prevalence was 0.27%. The PRUV incidence in the study population was 1/370 in the study population. No chromosomal anomaly was found in PRUV cases, but one case had echogenic intracardiac focus, one case had echogenic intestine, and one case had short nasal bone. When organ systems were evaluated, renal cyst was found in one case. No additional finding was found in one case. Conclusion PRUV is the most common form among fetal venous system anomalies. If detected, fetal examination is required in terms of the anomalies that may accompany. Chromosome analysis is not necessary if it is isolated, and it should be evaluated as a variant of normal anatomy.

Retroesophageal left brachiocephalic vein with an ectatic aberrant left subclavian artery: A rare double vascular loop.

We hereby present a rare variant course of left brachiocephalic vein coexisting with an aberrant right subclavian artery in a patient with a ventricular septal defect with pulmonary atresia. This case highlights the importance of CT angiography in diagnosing associated aortic arch and systemic venous anomalies in complex congenital heart diseases.

Incidence of systemic venous anomalies in tetralogy of Fallot on multidetector CT angiography: A retrospective single‐center study of 973 patients

The present study sought to estimate the incidence and evaluate the patterns and associations of systemic venous anomalies in patients with tetralogy of Fallot on multidetector computed tomography (CT) angiography. We retrospectively reviewed CT angiographies of 973 pediatric age group patients diagnosed with tetralogy of Fallot, performed at our institution between 1st January 2015 and 31st December 2019, for systemic venous drainage patterns. We also proposed a structured classification and reporting system on CT angiography which would simplify the characterization, classification, and communication of these anomalies. Variations in systemic venous drainage patterns were observed in 146 (15.01%) patients. The most commonly observed anomaly was persistence of left superior caval vein, which was seen in 112 (11.51%) patients, with the most prevalent pattern being the presence of bilateral superior caval veins with no intercommunicating vein (n = 85). The second most common anomaly observed was presence of anomalous brachiocephalic vein (39/973 [4.01%]). Patients with an anomalous course of brachiocephalic vein had significantly higher prevalence of a right aortic arch than those without (23/39 [58.97%] vs 188/934 [20.13%]; P < .0001). Similarly, a higher prevalence of pulmonary atresia was noted in patients with an anomalous course of brachiocephalic vein compared to those without (8/39 [20.51%] vs 80/934 [8.57%]; P = .0109). There is a high incidence of anomalies of superior caval veins and brachiocephalic vein in the presence of tetralogy of Fallot. With advancements in corrective cardiac surgery and use of intraoperative cardiopulmonary bypass, the presence of systemic venous anomalies assumes greater significance making preoperative identification of these anomalies imperative.

Agenesis of Ductus Venosus: A Case Series

Ductus venosus agenesis is a rare anomaly which is significantly associated with cardiac, extracardiac and chromosomal anomalies. The prognosis depends on its association with other congenital abnormalities independent of the type of shunt. The aim of this study was to evaluate the ductus venosus in 2nd trimester scans and to evaluate associated congenital malformations associated with agenesis of the ductus venosus and its feto-neonatal outcome. We performed a retrospective study for a 5 year period from November 2015 to November 2019. Of the 8 cases seen, 2 (25%) had an intrahepatic shunt and 6 (75%) presented with extrahepatic umbilical venous drainage. Agenesis of ductus venosus was associated in 5 cases (62%) with cardiac abnormalities, 6 cases (75%) with extracardiac anomalies and 3 (37%) cases with chromosomal abnormality. Our study showed poorer outcome in fetuses associated with both cardiac and extracardiac anomalies accounting for 75% of all cases whereas intrahepatic type with mild cardiomegaly had favourable pregnancy outcome. Agenesis of ductus venosus is significantly associated with fetal cardiac and extracardiac abnormalities independent of the type of umbilical venous drainage. Extrahepatic umbilical venous drainage even if isolated has the significant risk of congestive heart failure, hydrops and/or intrahepatic portal venous system anomalies. The intrahepatic type has better prognosis if associated with no or minor abnormality.

A CASE OF PERMANENT PACEMAKER IMPLANTATION FROM THE LEFT SIDE IN PERSISTENT LEFT SUPERIOR VENA CAVA – TECHNICAL CHALLENGES IN A RARE PRESENTATION

Abnormalities of the systemic venous system are not uncommon. Two well-known systemic venous anomalies are the persistent left superior vena cava (PLSVC) and interrupted inferior vena cava. PLSVC is usually detected and comes as a surprise for the cardiologist during cardiac permanent pacemaker implantation. We report the case of a 65-year-old male who had a history of recurrent syncope and diagnosed with a complete heart block 8 months back. For which, a single-chamber VVIR pacemaker was implanted through the right subclavian vein at Delhi. The patient was a known case of diabetes mellitus and had poorly controlled blood sugar levels. At present, the patient presented to our center with pacemaker pocket site infection and it was first decided to explain the right-sided permanent pacemaker, insert temporary pacemaker, and implant the new pacemaker on the left side through the left subclavian vein. In this case, during procedure, PLSVC detected accidentally; so, we discuss the challenges we faced during the right ventricular lead placement through PLSVC.