Vena Cava Anomalies Research Articles

Incidentally Detected Inferior Vena Cava Anomalies: 3 Case Reports

Objective: Inferior vena cava (IVC) anomalies are very rare vascular embryological variations, the incidence rate in the general population is 0.5%. IVC anomalies are usually asymptomatic and detected incidentally. The inferior vena cava is formed between the 6th and 8th weeks of intrauterine embryological development. IVC occurs due to the fusion of the supracardinal, postcardinal, and subcardinal veins during the embryological period. This union results from complex anastomosis of the embryological stage veins. During this time, various IVC anomalies may develop. IVC anomalies increase the risk of deep vein thrombosis in the lower extremity. Because of the increased risk of deep vein thrombosis in these patients, anti-embolism prophylaxis can be performed before the operation. Therefore, the risk of pulmonary embolism increases as well. The use of computed tomography has become more common nowadays. The detection rate of IVC anomalies has increased in examinations performed for other purposes. Case: IVC anomalies are important in terms of surgical interventions and the use of a vena cava filter. Knowing the existence of IVC anomalies can be crucial in preventing complications that may arise during surgical and radiological procedures. The objective of this study is to present three different cases with incidentally detected IVC anomalies.

Congenital venous anomalies associated with retrocaval ureter: evaluation using computed tomography.

Retrocaval ureter is a rare congenital anomaly resulting from anomalous development of inferior vena cava (IVC) and not from anomalous of the ureter. The anomaly always occurs on the right side due to regression of right supracardinal vein and persistence of right posterior cardinal vein. Retrocaval ureter tends to be associated with various vena cava anomalies because of the embryogenesis. We aimed to identify the prevalence of associated congenital venous anomalies (CVA) resulting from cardinal vein development in adults with retrocaval ureter using computed tomography (CT) images. The study included 22 adults with retrocaval ureter. We evaluated CT findings and determined the incidence of associated CVA using thin slice data sets from CT scanner with 64 or more detectors. We compared the prevalence of CVA in the retrocaval ureter group (mean age: 57 ± 19 years) and in the control group of 6189 adults with normal ureter (mean age: 66 ± 14 years). In the retrocaval ureter group, 4 (18.2%) adults had CVA including double IVC, right double IVC, preisthmic IVC with horseshoe kidney, and preaortic iliac confluence. One of 2 adults with preaortic iliac confluence had right double right IVC. In the control group, 49 (0.79%) adults had CVA including 37 double IVC, 11 left IVC, and 1 IVC interruption azygos continuation. Fifteen horseshow kidneys were found. The prevalence of associated CVA in the retrocaval ureter group was higher than that in the control group (p < 0.001). Retrocaval ureter is frequently associated with CVA. Various CVA with retrocaval ureter could happen because of abnormal development of not only the right posterior or supra cardinal vein but also other cardinal veins.

Prevalence of Inferior Vena Cava Anomalies and Their Significance and Impact in Clinical Practice

To evaluate the prevalence of inferior vena cava (IVC) anomalies in an asymptomatic healthy population and symptomatic patients. This was a multicentre retrospective observational study This study was conducted by reviewing the computed tomography (CT) images of 1 000 individuals from South Korea taken for a general medical check up (group A) and 1 000 patients from the USA who visited with various symptoms for which CT was required (group B). A third group of 800 patients with deep vein thrombosis (DVT) and CT from two US centres were used for comparison (group C). Twenty-eight patients with anatomical changes in the IVC due to intervention, extrinsic compression, trauma, other rare conditions, and poor image quality were excluded. The mean age ± standard deviation of each group was 50 ± 6, 54 ± 11, and 54 ± 15 years in groups A, B, and C, respectively. In group A, duplication was the most common anomaly (10 cases, 1.0%), followed by left sided IVC (four cases, 0.4%), hypoplasia (three cases, 0.3%), and megacava (one case, 0.1%). In group B, the most common IVC anomaly was hypoplasia in six cases (0.6%); duplication in three patients, left sided IVC in three patients, aplasia in two patients, web formation in two patients, and megacava in two patients. In group C, hypoplasia was the most common type (32 cases, 4.0%). The prevalence of hypoplasia in patients younger than 50 years of age was significantly higher compared with older patients (12.7% [14/110] vs. 5.3% [10/190]; p = .027). The risk of hypoplasia or aplasia was significantly higher in patients with DVT (odds ratio [OR] 17.032, 95% confidence interval [CI] 5.243 – 55.321), especially in patients with iliofemoral DVT (OR 34.211, 95% CI 10.323 – 113.378). In the normal group, IVC duplication was the most common variation, while hypoplasia was most common in patients with iliofemoral DVT, especially in younger ones.

Reintervention for Superior Vena Cava Obstruction After Heart Transplant

BackgroundChildren undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction. MethodsA retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021. Simple SVC anatomy included a single right SVC to the right atrium or bilateral SVCs with a left SVC to an intact coronary sinus, without prior superior cavopulmonary connection. Presence of anomalous SVC anatomy, superior cavopulmonary connection, or previous atrial switch operation defined complex anatomy. Reconstructive strategies included atrial anastomosis; direct SVC-to-SVC anastomosis; and augmented SVC anastomosis using innominate vein, patch, cavopulmonary connection, or interposition graft. The primary outcome was reintervention for SVC obstruction. ResultsOf 288 patients, pretransplant diagnoses included congenital heart disease (n = 155 [54%]), cardiomyopathy (n = 125 [43%]), and other (n = 8 [3%]). Most (n = 208 [72%]) had simple SVC anatomy compared with complex SVC anatomy (80 [28%]). Reintervention for SVC obstruction occurred in 15 of 80 (19%) with complex anatomy and 1 of 208 (0.5%) with simple anatomy (P = .0001). Reintervention was more common when innominate vein or a patch was used (9/25 [36%]) compared with an interposition graft (1/7 [14%]) or direct anastomosis (6/82 [7%]; χ2 = 13.1; P = .001). Most reinterventions occurred within 30 days of OHT (14/16 [88%]). ConclusionsPatients with complex SVC anatomy have a higher rate of reintervention for SVC obstruction after OHT compared with those with simple SVC anatomy. In cases of complex SVC anatomy, interposition grafts may be associated with less reintervention compared with complex reconstructions using donor tissue.

Deep Venous Thrombosis and the Unanswered Questions Surrounding Inferior Vena Cava Anomalies

The study by Kim et al. is a multicentre, observational, retrospective study conducted in the United States and South Korea.1 The authors investigated the difference in prevalence of inferior vena cava (IVC) anomalies in patients diagnosed with deep venous thrombosis (DVT) vs. those who had computed tomography (CT) scans for diagnostic purposes, and a control group who had CT scans for health screening purposes. The study concluded that the occurrence of IVC anomalies was 1.8% in the control group and 6.3% in the DVT group.

Prevalence of Inferior Vena Cava Anomalies and Their Significance and Impact in Clinical Practice

To evaluate the prevalence of inferior vena cava (IVC) anomalies in an asymptomatic healthy population and symptomatic patients. This was a multicentre retrospective observational study This study was conducted by reviewing the computed tomography (CT) images of 1 000 individuals from South Korea taken for a general medical check up (group A) and 1 000 patients from the USA who visited with various symptoms for which CT was required (group B). A third group of 800 patients with deep vein thrombosis (DVT) and CT from two US centres were used for comparison (group C). Twenty-eight patients with anatomical changes in the IVC due to intervention, extrinsic compression, trauma, other rare conditions, and poor image quality were excluded. The mean age ± standard deviation of each group was 50 ± 6, 54 ± 11, and 54 ± 15 years in groups A, B, and C, respectively. In group A, duplication was the most common anomaly (10 cases, 1.0%), followed by left sided IVC (four cases, 0.4%), hypoplasia (three cases, 0.3%), and megacava (one case, 0.1%). In group B, the most common IVC anomaly was hypoplasia in six cases (0.6%); duplication in three patients, left sided IVC in three patients, aplasia in two patients, web formation in two patients, and megacava in two patients. In group C, hypoplasia was the most common type (32 cases, 4.0%). The prevalence of hypoplasia in patients younger than 50 years of age was significantly higher compared with older patients (12.7% [14/110] vs. 5.3% [10/190]; p= .027). The risk of hypoplasia or aplasia was significantly higher in patients with DVT (odds ratio [OR] 17.032, 95% confidence interval [CI] 5.243 - 55.321), especially in patients with iliofemoral DVT (OR 34.211, 95% CI 10.323 - 113.378). In the normal group, IVC duplication was the most common variation, while hypoplasia was most common in patients with iliofemoral DVT, especially in younger ones.

Prevalence of Congenital Anomalies of the Inferior Vena Cava: CT Assessment in Thai Patients

Objective: To determine the prevalence of inferior vena cava (IVC) anomalies in Thai patients who underwent contrast-enhanced computed tomography (CT) of the abdomen. Materials and Methods: Two radiologists retrospectively and independently reviewed the contrast-enhanced abdominal CT examinations in 1,429 Thai patients between August 1, 2018 and January 25, 2019 who met the inclusion criteria. Patients were included, if (a) their CT showed well visualized IVC, renal veins, and right ureter that were not obliterated by tumor, cyst, fluid collection, or intraperitoneal free fluid, (b) they had not undergone previous abdominal surgery that altered anatomical configuration of the IVC, renal veins, and right ureter. The presence of all IVC anomalies were recorded. Results: Among the 1,429 studied patients, 678 were male (47.4%) and 751 were female (52.6%). The prevalence of IVC anomalies was 3.5%. Five types of IVC anomalies were presented. The most common was circumaortic left renal vein in 24 patients or 48.0% of all IVC anomalies and 1.7% of the study population, followed by retroaortic left renal vein in 15 patients or 30.0 % of all IVC anomalies and 1.0% of the study population. Other IVC anomalies included double IVC, left IVC, and retrocaval ureter at 0.5%, 0.2%, and 0.1% of the study population, respectively. Conclusion: The prevalence of IVC anomalies in the present study differed from the previous studies conducted in other countries, which may be attributable to differences in race and ethnicity. Awareness of these anomalies is essential when evaluating routine CT examinations in asymptomatic patients. Their presence should be carefully noted in radiology reports to avoid anomaly-related complications. Keywords: Prevalence; IVC anomalies; Circumaortic left renal vein; Retroaortic left renal vein; Double IVC; Left IVC; Retrocaval ureter

English

Double inferior vena cava is a rare anomaly with incidence rate of 0.2 - 3 %. It occurs due to non-regression of both right and left supracardinal veins during embryonic development. Here, we present a case with double inferior vena cava diagnosed in a patient who underwent routine computed tomography for abdominal pain. In our case, both right and left inferior vena cava are of same caliber and we believe that duplication of IVC in our case was a result of nonregression of anastomosis between left supra subcardinal, posterior subcardinal and intersubcardinal veins resulting in persistence of left subcardinal vein. The knowledge of this anatomical variation is clinically important during retroperitoneal surgeries and vascular and radiological interventional procedures. Inferior vena cava anomalies are rare and incidentally found in asymptomatic patients who undergo radiological imaging for some other diseases. The reported incidence of duplicated inferior vena cava is 0.2 to 3 %. Among all the inferior vena cava anomalies, inferior vena cava duplication and left inferior vena cava are most commonly found. 1,2 Vascular anomalies often get encountered in computed tomography (CT) scans of abdomen and pelvis obtained with contrast injection. Familiarity with these variations is essential for correct interpretation.3 Here, we present a case of duplication of inferior vena cava with left IVC draining into right IVC at upper border of L2 vertebral level.

Coronary sinus atrial septal defects in adults over the past 20\u2009years at new Tokyo hospital: case series

BackgroundAn isolated coronary sinus (CS) atrial septal defect (ASD) is defined as a CS unroofed in the terminal portion without a persistent left superior vena cava or other anomalies. This defect is rare and part of the wide spectrum of unroofed CS syndrome (URCS). Recently, several reports have described this finding. The database of New Tokyo Hospital was searched to determine the incidence of this defect. Additionally, to raise awareness of this condition, the findings from five patients with CS ASD who underwent surgical repair at New Tokyo Hospital are discussed.Case presentationThe patients were three women and two men with an age range of 63–77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with different combinations and degrees of mitral, tricuspid, and aortic valve involvement. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms.ConclusionsCS ASD (Kirklin and Barratt–Boyes type IV URCS) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries at New Tokyo Hospital from 1999 to 2019. At New Tokyo Hospital, cardiac surgery is performed mainly for patients with acquired cardiac disease, and CS ASD is rare. Early diagnosis is important, as well as early surgical repair in symptomatic patients, especially those with blood access shunts, which may overload the heart. The case of a poor prognosis in this series is noteworthy, as similar cases have not been reported previously.

Atrial Septal Defect Closure via Left Subclavian Vein Approach in a Patient With Absent Inferior Vena Cava.

Without the femoral venous approach, transcatheter closure of an atrial septal defect is challenging. We performed percutaneous closure via the left subclavian vein in a patient with absence of the inferior vena cava with azygos continuation. Considering that inferior vena cava anomalies are not extremely rare among those with congenital heart disease, the left subclavian vein approach can be an alternative to the femoral approach.

Inferior vena cava anomalies: review and surgical considerations

Angiología es una revista científica bimensual editada desde 1949. Está dedicada al estudio de las enfermedades vasculares, incluyendo todos los aspectos de la medicina vascular, el diagnóstico no invasivo, el tratamiento quirúrgico tradicional arterial y venoso, así como el mínimamente invasivo y/o endovascular.

Clinical and procedural implications of congenital vena cava anomalies in adults: A systematic review

BackgroundAlthough congenital vena cava (CVC) anomalies in adults have implications for surgical and radiological interventions, the literature is scare and disparate. The aim of this systematic review was to assess cardiovascular clinical and procedural implications of CVC anomalies in adults without congenital heart disease. Methods and resultsWe searched PubMed and EMBASE from database conception through October 2018 for English-language studies describing the epidemiology of CVC anomalies or their clinical or procedural implications in humans. Two independent reviewers screened 7093 records and identified 16 relevant studies. We found two major implications of CVC anomalies: 1) congenital inferior vena cava (CIVC) anomalies are associated with a 50–100-fold higher risk of deep venous thrombosis, particularly among younger patients, and 2) persistent left superior vena cava (PLSVC) is associated with a 2–3-fold higher risk of supraventricular arrhythmias. PLSVC also poses technical challenges to cardiovascular electronic device implantation, requiring alterations in surgical approach and lengthening procedure and X-ray exposure times. Due to the large disparity in reported prevalence rates of CIVC anomalies, we performed a meta-analysis of CIVC anomaly prevalence including 8 studies, which showed a weighted prevalence of 6.8% (95% CI, 4.5–9.2%). ConclusionThese findings challenge the notion that CVC anomalies are rare and asymptomatic in adults. Rather, the literature indicates that CVC anomalies are not uncommon and have important clinical and procedural implications. To further understand the prevalence and implications of CVC anomalies, a robust US population-based study and nationwide registry is warranted in the current era of venous interventions.

SUCCESSFUL PERFORMANCE OF CARDIAC ELECTROPHYSIOLOGICAL PROCEDURES VIA A CONGENITAL INFERIOR VENA CAVA ANOMALY- REPORT OF 2 CASES

Background: Femoral vein access is the preferred approach for advancing multiple catheters via the inferior vena cava (IVC) to the heart during routine cardiac electrophysiology study and catheter ablation. Compared to acquired venous abnormalities, congenital inferior vena cava anomalies are encountered rather infrequently in adult electrophysiology procedures and their presence may pose technical procedural challenges. Presentation: We describe 2 cases in which we were able to successfully perform cardiac electrophysiological procedures in the presence of a complex congenital venous anomaly, the left sided IVC. First case was 30 year old gentleman presented with history of recurrent episodes of supraventricular tachycardia terminated with AV nodal blocking agents. Second case was 21 year old boy with WPW syndrome and recurrent supra-ventricular tachycardia. Diagnosis and Management: We managed to pass the EP catheters in both cases with a bit difficulty and angulation, while given I/V heparin to reduce the risk of thrombosis and confirmed the position of catheters via subclavian venous placement of a guidewire. In first case, typical AV node reentry tachycardia was induced which was mapped and ablated in the slow pathway region. In second case, right posterior accessory pathway was ablated at 6 0’clock. CT angiography of the abdominal veins was performed which confirmed the finding of left sided IVC. Follow-up and Outcomes: Abnormalities of the IVC are relatively uncommon. But it is an important condition that may be encountered by electrophysiologist. Catheter ablation of the slow AV nodal pathway and right posterior accessory pathway was safely and successfully performed with this unusual venous anomaly. Keywords: SVT, LEFT IVC, RFA

Massive Inferior Vena Cava Thrombosis Associated With Retroperitoneal Venolymphatic Malformation: Use of Intravascular Ultrasound for Diagnosis and Management

Inferior vena cava (IVC) anomalies are uncommonly described. Many are associated with a higher rate of thromboembolic complications. Here we report a case of IVC thrombosis in a 20-year-old woman with a large isolated retroperitoneal venolymphatic malformation (VLM) with IVC envelopment in which a

Inferior vena cava calcification, a possible link with recurrent deep venous thrombosis and pulmonary embolism: a case study and review of literature.

Inferior vena cava (IVC) anomalies have been reported to have an association with deep venous thrombosis of the lower limbs. It is, therefore, necessary to study the IVC in recurrent cases of unprovoked deep venous thrombosis (DVT) and/or pulmonary embolism (PE), where all other causes have been excluded. We report a case of a 65-year-old male, who had recurrent episodes of DVT in the past 5 years; some of which associated with PE of unknown cause. CT thorax abdomen and pelvis did not find an obvious cause for the DVT and/or PE, however, it did highlight a diffuse calcification of the IVC. Only a few cases of calcification of the IVC have been reported in literature, and a number of them have been associated with clot formation and PE. We speculate that, as in other anomalies of the IVC, calcification of the IVC might slow the blood flow, and thus predispose to DVT and/or PE. Our opinion is that in all cases of unexplained DVT and/or PE, a careful examination of the IVC should be performed. Furthermore, when this condition is present, other risk factors for hypercoagulability should be avoided and anticoagulant therapy should be considered.

Techniques of adrenal venous sampling in patients with inferior vena cava or renal vein anomalies.

To review the techniques and technical success rate of adrenal venous sampling (AVS) in patients with inferior vena cava (IVC) or renal vein anomalies. The techniques and success rate of AVS in 15 patients with anomalies [8 with double IVC (dIVC), 3 with left IVC (ltIVC), 2 with retroaortic left renal vein (LRV), and 2 with circumaortic LRV] underwent AVS was retrospectively reviewed. Among 11 patients with IVC anomalies, the success rates for sampling the right and left adrenal veins (RAV and LAV) were 81.8 and 90.9%, respectively. In dIVC, the LAV was selected using the following four methods: approaching through the right IVC from the right femoral vein, flipping the LAV catheter tip in the LRV (n = 4) or the interiliac-communicating vein (n = 1), or through the ltIVC from the right (n = 1) or left (n = 2) femoral vein. Among the four patients with LRV anomalies, the success rate was 100% for each adrenal vein. AVS can be successfully performed in patients with anomalies. The key to technical success is understanding the venous anatomy based on pre-procedural CT images and choosing appropriate methods.

Vena cava anomalies in thoracic surgery

BackgroundVena cava anomalies are a rare group of anatomical variations due to an incorrect development of the superior or inferior vena cava during fetal life. They generally show no clinical relevance and the diagnosis is done due to the association with congenital heart diseases in most of cases. However, preoperative identification of these anomalies is mandatory for surgeons to proper surgical planning. If not recognized, lethal complications may occur, as already reported in literature.Case presentationWe report a case series of three different unidentified vena cava anomalies in patients undergoing lung resection. These unrecognized anomalies led to minor complications in two cases and required an accurate intraoperative evaluation in another.A careful retrospective evaluation of preoperative radiological images showed the anomalies.ConclusionsA careful evaluation of the vena cava anatomy at pre-operative imaging is mandatory for thoracic surgeons to properly plan the surgery and avoid complications.

Inferior vena cava anomalies: Incidental finding in two cases during computed tomography scan

Inferior vena cava anomaly is usually asymptomatic. It is rare and commonly detected while performing abdominal imaging for other clinical situations. The anomalies can mimic normal anatomical structures. Thus, the identification of the anomalies is important to avoid complication during abdominal procedures. We report two cases of inferior vena cava anomalies found during Computed Tomography scan of our elective cases.

Congenital anomalies of the IVC-embryological perspective and clinical relevance.

With the increased use of cross-sectional imaging, systemic venous anomalies are more frequently being recognized in asymptomatic patients. Accurate characterization of systemic venous anomalies plays a major role in the appropriate selection of a surgical approach or interventional procedure. In this article, we review common and uncommon inferior vena cava (IVC) anomalies. We describe the embryological basis and clinical implications of these anomalies, particularly from an interventional radiology perspective. We also discuss the complications and treatments of these anomalies.

Complicated IVC anomalies: Are they more common than we thought? An experience of 100 MDCT venography examinations

ObjectiveThe aim of this study was to monitor the incidence of complicated inferior vena cava (IVC) anomalies and evaluate the role of Multidetector Computed Tomographic Venography (MDCTV) in diagnosis and assessment of associated venous collaterals, lower limb deep venous thrombosis (DVT) or varicose veins (VV). Patients and methodsDuring two years duration 100 patients with clinical history and complains suggesting of DVT or VV were prospectively evaluated after performance of MDCTV examination. The images obtained were interpreted and reconstructed using dedicated software and work stations. Results were correlated with Color Doppler Ultrasound (CDUS) findings. ResultsOut of 100 cases, 9 cases (9%) were diagnosed to have complicated IVC anomalies while 91 cases (91%) had either well developed IVC or common anatomical variations. 6 cases (66.7%) had complicated IVC anomalies and 3 cases (33.3%) had associated complicated common iliac veins (CIV) anomalies. 8 cases (88.9%) had associated DVT and all cases (100%) had bilateral VV. 2 cases (22.2%) had associated varicocele and 1 case (11.1%) had associated KILT syndrome. ConclusionMDCT venography examination has a major role in diagnosis of complicated IVC anomalies and detection of associated venous collaterals, lower limb DVT or VV.