Inferior Vena Cava Abnormalities Research Articles

Prenatal Ultrasound in Fetal Inferior Vena Cava Abnormalities: Image Characteristics and Clinical Relevance

Background: During the embryonic stage, the inferior vena cava (IVC) is an important conduit for the hepatic vein and ductus venosus to drain into the right atrium. For fetuses with IVC abnormalities, the prognosis may be favorable if the condition is not complicated with other malformations, but would be poor if atrial isomerism coexists. In severe cases, edema, intrauterine fetal death, and atrioventricular block may occur. Therefore, comprehensive prenatal ultrasound that provides detailed information about IVC abnormalities may be clinically significant. Methods: A total of 180 fetuses diagnosed with IVC anomalies via prenatal ultrasound at Hospital of Chengdu University of Traditional Chinese Medicine and Sichuan Provincial Maternity and Child Health Care Hospital from January, 2017 to December, 2022 were included in this study. Their ultrasound image characteristics, associated intra- or extracardiac malformations and pregnancy outcomes were retrospectively analyzed. Results: Among the 180 fetuses, 59 cases were diagnosed with interrupted IVC (53 cases with the interruption of the hepatic segment of the right IVC and 6 cases with the interruption of the entire right IVC), 1 case was diagnosed with the stenosis of the hepatic segment of the right IVC, 90 cases were diagnosed with left sided IVC, 29 cases with double IVC, and 1 case with abnormal connection of the IVC to the left atrium. Moreover, 33 cases had intracardiac malformations and 36 cases had extracardiac malformations. Pregnancy outcomes: 160 fetuses were live born, and their prenatal ultrasound diagnoses were confirmed by computed tomography (CT)/magnetic resonance imaging (MRI) or surgery; the remaining 20 fetuses were terminated due to serious malformations, and their prenatal ultrasound diagnoses were confirmed by pathologic examination. Conclusions: Prenatal ultrasound can clearly reveal the fetal IVC abnormalities and the associated intra- or extracardiac malformations. For suspected cases, attention should be focused on prenatal ultrasound examinations in order to obtain valuable information for prenatal consultation and subsequent procedures and care.

KILT syndrome - kidney and inferior vena cava abnormalities with leg thrombosis

KILT syndrome - kidney and inferior vena cava abnormalities with leg thrombosis

A rare case of KILT syndrome in Ireland: A case report

Venous thromboembolism (VTE), comprising deep vein thrombosis (DVT) and pulmonary embolism (PE), is associated with significant morbidity and mortality. Congenital absence of the inferior vena cava (IVC) is reported in current literature as a rare risk factor for DVT. The presence of kidney and IVC abnormalities on computed tomography together with lower limb thrombosis is collectively referred to as KILT syndrome (Kidney and IVC Abnormalities with Leg Thrombosis), an extremely rare cause of extensive bilateral lower limb DVT.We present a case of KILT syndrome, diagnosed for the first time after the age of 60 years and the first case reported in the Irish population. The patient presented to the emergency department with unilateral limb pain with a prior history of provoked DVT. Clinical gestalt, a high Wells Score, and raised D-Dimer strongly supported a diagnosis of DVT. Lower limb duplex ultrasound confirmed bilateral external iliac and femoral vein thrombus, with computed tomography venography revealing an absent infrarenal IVC and atrophy of the left kidney. The medical team subsequently admitted the patient for therapeutic anticoagulation and investigation. After review by haematology, the patient was discharged on an indefinite duration of anticoagulation with follow-up with our coagulation services. In any patient with bilateral lower limb DVT, comprehensive assessment and investigation, with particular emphasis on imaging, is imperative to exclude rare associated conditions.

Spectrum of thoracic systemic venous abnormalities using multidetector computed tomography

BackgroundAnomalies of the thoracic systemic venous return vary widely and range from those with completely normal physiology to severe right to left shunting thus requiring surgical correction. The aim of the study is to enhance the awareness of multidetector computed tomography (MDCT) role in evaluation of systemic venous abnormalities, and be familiar with the imaging characteristics of even the extremely rare abnormalities.ResultsAmong 270 examined patients, 15.19% had systemic venous abnormalities. Inferior vena cava (IVC) congenital anomalies accounted for (24.4%) of the detected abnormalities (prevalence: 3.7% among the studied population) where IVC interruption with azygos continuation was the most common detected IVC abnormality accounting for 17.7% of the detected abnormalities (prevalence: 2.6%), while IVC thrombosis accounted for 21.9% (prevalence: 3.3%). Persistent left sided superior vena cava (SVC) accounted for 14.6% of the detected abnormalities (prevalence: 2.2%), while SVC syndrome represented 19.5% (prevalence: 2.9%) and SVC aneurysm represented 2.4% (prevalence: 0.37%). Retroaortic brachiocephalic vein (BCV) and BCV thrombosis accounted for 7.3% each (prevalence: 1.1). Finally, persistent levo-atrial cardinal vein represented 2.4% of the detected abnormalities (prevalence: 0.37%).ConclusionsMDCT is a non-invasive modality that can provide detailed information about the systemic thoracic veins before surgical or interventional procedures, especially in patients with congenital anomalies.

Abstract 10682: Peripartum Myocardial Strain Analysis: A Marker for Adverse Cardiovascular Outcomes?

Introduction: Hypertensive disorders of pregnancy (HDP) contribute significantly to adverse cardiovascular (CV) outcomes in peripartum women, and echocardiography helps stratify risk and guide management. Global longitudinal strain (GLS), a marker of left ventricular (LV) systolic function, has not been well-characterized in peripartum women with or without HDP. Hypothesis: We hypothesized that GLS is abnormal in women with prior HDP and may predict adverse CV outcomes. Methods: We retrospectively analyzed 141 women with echocardiograms performed during the peripartum period (6 weeks antepartum to 8 weeks postpartum). We investigated relationships between adverse CV outcomes and clinical and echocardiographic variables, including peak systolic GLS. Prior HDP was defined as a preceding diagnosis of gestational hypertension (HTN), chronic HTN or preeclampsia. Results: Women with prior HDP (72/141, 51%) were older (35 vs 32 years), more often obese (61% vs 34%) and had higher mean arterial pressure (96 vs 82 mmHg) compared to those without prior HDP (p<0.01 for all). Women with prior HDP had greater LV thickness (1.0 vs 0.9 cm) and more frequently abnormal inferior vena cava (IVC) size and collapsibility (p<0.01 for all), although median LV ejection fraction (64% vs 65%) and GLS (-19.6 vs -20.2) were similar. During follow-up of 2.5 years, women with prior HDP had more CV-related emergency department (ED) visits (36% vs 16%, p<0.01) and hospitalizations (32% vs 12%, p<0.01). Using multivariable modeling, left atrial area (LAA) and IVC size better predicted CV-related ED visits and hospitalizations when compared to age, obesity and history of gestational HTN ( Figure ). Conclusions: Although GLS did not predict CV outcomes, abnormal LAA and IVC suggest elevated left and right atrial pressure is related to HDP and CV outcomes. Further study of LV diastolic or right ventricular dysfunction may elucidate mechanisms contributing to adverse outcomes in this population.

Vena Cava Atresia and Deep Vein Thrombosis: A Case Report and Systematic Review.

Congenital anomalies of the vena cava often go unnoticed, and their incidental finding is frequent in imaging tests performed for another cause in asymptomatic patients. However, an association with the development of thromboembolic disease has been described, mainly in young patients. We report the case of a young woman with deep vein thrombosis associated with vena cava atresia. We conducted a search of the PubMed/Medline database. The search identified 17 articles, of which 5 were selected for extraction and data analysis. Twelve studies were excluded because they failed to match the main criteria. We identified six new cases of deep vein thrombosis associated with vena cava abnormalities, with a mean age of 42.5 years; 83.3% were male. Regarding clinical manifestations, all patients presented as deep vein thrombosis, one case of recurrence, and another case associated with the kidney and inferior vena cava abnormalities with leg thrombosis syndrome. Only one patient had a target triggering factor (cholecystectomy postoperative). The thrombophilia study was negative in all cases and none of the patients died. Treatment included enoxaparin and vitamin K antagonists. This is the first study to report on a systematic review of vena cava atresia associated with deep vein thrombosis in Spain. It shows that in this region, the disease affects young population-even in the absence of risk factors-and is linked to a low mortality. The most frequent presentation form was deep vein thrombosis. Therefore, congenital abnormalities of the vena cava should be suspected in young patients with thromboembolic disease, due to their implications regarding to the duration of anticoagulant treatment, as well as their possible association with other prothrombotic factors.

Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Answers.

Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Answers.

Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Questions.

Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Questions.

INFERIOR VENA CAVA AGENESIS: ANATOMICAL VARIANT AS CAUSE OF RECURRENT DVT

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Abnormalities of the inferior vena cava (IVC) are rare causes of deep venous thrombosis (DVT). The clinical presentation is often similar to that of an inherited hypercoagulabe condition. CASE PRESENTATION: A 27-year-old man with a past medical history of premature birth presented to the emergency department (ED) with left lower extremity (LLE) tightness and pain for one day. He had no history of recent travel and family history was negative for clotting disorders. Ultrasound (US) of the left leg showed an occlusive thrombus from the left common femoral to the left popliteal vein. The patient was discharged from the ED on rivaroxaban. Two weeks later, he presented to the ED again with progressive LLE swelling despite medication compliance. Physical exam was negative for testicular masses. Interventional Radiology (IR) performed a LLE venogram, which showed thrombus extention to the common iliac vein. US-assisted catheter thrombolysis (EKOS) was initiated. Heparin infusion was started and the patient was transferred to the intensive care unit. Repeat visualization the following day revealed improved clot in the left femoral and external iliac veins and a chronically thrombosed left common iliac vein. LLE pain and swelling improved and EKOS was discontinued twenty-four hours later. Hypercoagulability work-up was negative except for lupus anti-coagulant, the significance of which was difficult to determine, given prior anticoagulant exposure. On discharge, the patient was transitioned back to rivaroxaban. Five days later, the patient presented to the ED again with worsening LLE pain and swelling after initial improvement. Repeat venous US showed occlusive thrombus throughout much of the left femoral vein with only some flow at the distal aspect. An inferior vena cavogram revealed agenesis of the IVC with azygous continuation. CT angiogram of the abdomen and pelvis confirmed an occluded infra-renal IVC secondary to atresia. Patient was discharged on indefinite rivaroxaban therapy. DISCUSSION: This patient presented with an unprovoked DVT involving the common iliac vein that extended despite appropriate anticoagulation. Eventually, a venogram revealed agenesis of the IVC as the predisposing risk factor. In this condition, venous stasis presumably leads to increased risk of clotting. While bilateral DVTs are rare in the general population (approximately 10% of cases), they have been described in up to three quarters of patients with IVC abnormalities. This patient however, had extensive unilateral clot burden, usually seen in more distal anatomical abnormalities, such as May-Thurner syndrome. His premature birth likely predisposed him for an anatomical malformation. CONCLUSIONS: Unprovoked DVT in a young patient, recurrent DVTs despite medication compliance, and occlusion of the iliac veins, should raise suspicion for anatomical abnormalities, such as IVC atresia. Reference #1: 1. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol. 2001;114(4):878. Reference #2: 2. Ruggeri M, Tosetto A, Castaman G, Rodeghiero F. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet. 2001;357(9254):441. Reference #3: 3. Hamoud S, Nitecky S, Engel A, Goldsher D, Hayek T. Hypoplasia of the inferior vena cava with azygous continuation presenting as recurrent leg deep vein thrombosis. Am J Med Sci. 2000;319(6):414. DISCLOSURES: No relevant relationships by Amy Cohen, source=Web Response No relevant relationships by David Cohen, source=Web Response No relevant relationships by Aaron Dickinson, source=Web Response No relevant relationships by Mouhanned Eliliwi, source=Web Response No relevant relationships by Jennifer Meyfeldt, source=Web Response

1108 Inferior vena cava diversion into the left atrium after surgical repair of atrial septal defect: a rare case of chronic hypoxemia

Abstract Abnormal inferior vena cava (IVC) drainage into the left atrium (LA) is an infrequent complication after surgical closure of an atrial septal defect (ASD). We present the case of a 45-year-old woman, with surgical closure of an ASD 8 years before, who consulted for exertional dyspnea and cyanosis. Transthoracic and transesophageal echocardiography showed no residual shunt after contrast administration in both arms, and thoracic CT scan and pulmonary ventilation / perfusion gammagraphy were normal. In the presence of signs strongly suggesting a right-to-left shunt, we decided to complete the study with a CMR,which revealed an anomalous systemic drainage with IVC draining into the LA with right-to-left shunt. Surgical repair of the abnormal IVC drainage was successfully performed. During the intervention cardiac surgeons found a repaired ASD located in the lower portion of the interatrial septum, a large Eustachian valve, which are anatomical features which can sometimes lead to an erroneous suture causing the drainage of the IVC towards the LA. In conclusion, it is essential to carry out an extensive search for possible residual anatomical shunts in patients who undergo surgery and develop hypoxia. The reported case underlines the importance of being aware of this possible complication of the closure of an ASD and highlights that the absence of passage of agitated saline contrast to left chambers with conventional injection by the arms does not rule out any residual shunt, recommending its administration by the lower limbs, as well as the CMR for the definitive diagnosis. Abstract 1108 Figure. CMR imaging 3D reconstruction

Ultrasound diagnosis of left inferior vena cava and double inferior vena cava in fetus

To evaluate the application of ultrasonography in prenatal diagnosis of left inferior vena cava and double inferior vena cava in fetus. The clinical data and ultrasonographic findings of the fetuses with left inferior vena cava (18 cases) or double inferior vena cava (16 cases) were retrospectively analyzed. The ultrasonographic images of left inferior vena cava showed that in the transverse view of the fetal upper abdomen the inferior vena cava and abdominal aorta were in the normal position; below the level of the hilum, the inferior vena cava was located behind the left side of the abdominal aorta; at the level of the hilum, it crossed the front of the abdominal aorta and run diagonally to the upper right, forming the right inferior vena cava and finally entered into the right atrium. The ultrasonographic images of double inferior vena cava showed that in the transverse view of the fetal lower abdomen, in front of spine there were three transections of blood vessels; in coronal plane of abdomen, the veins run on both sides of the abdominal aorta and entered to the iliac vein of the same side. In 34 cases of abnormal inferior vena cava, there were 17 cases complicated with other system abnormalities, including 13 cases of cardiac anomalies. The left inferior vena cava and double inferior vena cava have characteristic imaging findings, and prenatal diagnosis can be made with ultrasonography. This type of congenital deformity is frequently complicated with other system abnormalities, which should be excluded in fetus, especially for heart system.

Kidney and inferior vena cava abnormalities with leg thrombosis syndrome in a 22-year-old

Kidney and inferior vena cava abnormalities with leg thrombosis syndrome in a 22-year-old

A Case Report: Hanging hepatoatrial anastomosis in cadaveric donor liver transplantation for a Budd-Chiari Syndrome Patient

Introduction: The liver transplantations for Budd-Chiari syndrome(BCS) patients are challenging for variety of reasons. There is no standardized procedure for the reconstruction of the diseased hepatic vein or inferior vena cava(IVC) segments. The underlying hypercoagulable state or hematologic disorders in BCS patients may, also, raise problems during postoperative period. Although anastomotic failure of hepatic vein is an unusual complication in adult orthotopic liver transplantation(OLT), pre-existing inferior vena cava abnormality, such as in BCS, may contribute to the increased rate of the hepatic outflow complications. Technical adjustments are to be made in such cases. Method: case: A fifty-one-year-old male patient was admitted to Ajou University Hospital for impending liver failure. He was, previously diagnosed with Budd-Chiari syndrome. The preoperative CT scan revealed extensive IVC trhrombosis from the right atrium inlet to both common iliac veins. Result: A statisfactory result was obtained by hanging hepatoatrial anastomosis during cadaveric donor liver transplantation.. For graft implantation, the upper cuff of the donor IVC was sutured directly to the right atrium and the lower cuff was closed by oversewing. No subsequent complication relevant to this vascular anastomosis was observed during six months of follow-up period. Conclusion: The modification of hepatic outflow reconstruction during liver transplantation that we have named a hanging hepatoatrial anastomosis can be tolerated in such patients with effective collateral vessel system.

Rare case of a kidney and inferior vena cava abnormalities with extensive lower extremity deep vein thrombosis in a young healthy male.

Kidney and inferior vena cava (IVC) abnormalities with extensive deep vein thrombosis (DVT) is a very rare cause of DVT and has a diverse clinical presentation. Computed tomography (CT) angiography is the gold standard for diagnosis and treatment including thrombectomy, thrombolysis and systemic anticoagulation. We present a rare case of active young healthy male admitted with acute onset of right lower extremity pain and swelling who was found to have extensive DVT on doppler ultrasound. CT abdomen showed extensive clot burden involving right common femoral vein extending into internal and external iliac veins associated with IVC hypoplasia and hypoplastic left kidney. Patient underwent urgent thrombectomy, catheter directed thrombolysis and was discharged home in stable condition on oral anticoagulation.

Pulsatile Glenn as long-term palliation for single ventricle physiology patients.

There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. A retrospective review was performed. Study performed at a single pediatric hospital. All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses. Seventy-eight patients underwent pulsatile Glenn at age 9months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7years [interquartile range, 3-11], oxygen saturation 83%±4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002). Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.

Hanging Hepato-Atrial Anastomosis in Deceased-Donor Liver Transplantation for Budd-Chiari Syndrome With Extensive Vena Cava Obliteration: A Case Report

IntroductionAlthough outcomes of liver transplantation (LT) have improved as the result of progress in surgical procedures, a failure to restore sufficient graft outflow may yield fatal consequences including graft dysfunction and even graft loss to date. In particular, patients with pre-existing obliterated venous drainage, such as those with Budd-Chiari syndrome (BCS), are at high risk of having venous complications followed by conventional LT. In selected cases, the transplant surgeons are compelled to modify the surgical procedures of LT from the conventional procedure. MethodsWe describe an LT performed in a BCS patient with complete inferior vena cava (IVC) obstruction. A procedure that we named “hanging hepato-atrial anastomosis” was performed, in which 2 major modifications were made. One modification was the dissection of the lower inlet of the right atrium by use of a trans-abdominal approach and hepato-atrial anastomosis. This was performed by exposing the thoracic IVC through a trans-abdominal approach. The other modification was the manufacture of a blind pouch from the graft's infra-hepatic IVC without anastomosis. ResultsModifications were made possible as the result of meticulous examination of the patient's vascular anatomy before the operation. Fortunately, the patient had a heavy network of pre-vertebral veins that drained blood from the lower extremity and both kidneys to the azygos–hemi-azygos veins. ConclusionsWe learned that a meticulous assessment of vascular anatomy and complete understanding of hemodynamics are the keys to the successful LT for BCS in patients with extensive IVC abnormality. Thoracotomy may not be necessary to explore thoracic IVC when performing hepato-atrial anastomosis in LT for BCS.

Deep Venous Thrombosis Associated With Inferior Vena Cava Abnormalities And Hypoplastic Kidney In Siblings

Abstract Congenital inferior vena cava anomalies have a reduced frequency in general population, many times being an asymptomatic finding. Patients caring such anomalies are at risk to develop deep vein thrombosis. In this paper, we present 2 siblings with deep venous thrombosis and inferior vena cava abnormalities, with a symptomatic onset at similar age. The inferior vena cava abnormality was documented by an angio-CT in each case. The thrombophilic workup was negative. Patients were treated with conservative therapy: low molecular weight heparin anticoagulants converted later to oral anticoagulant with resolution of symptoms and disappearance of the thrombus. Finally, in the absence of any risk factor in a young patient admitted with deep vein thrombosis investigations to exclude inferior vena cava anomalies are mandatory.

СONGENITAL ABNORMALITIES OF THE INFERIOR VENA CAVA AND ITS TRIBUTARIES: THEIR RADIOLOGICAL DIAGNOSIS AND CLINICAL SIGNIFICANCE

Abnormalities of the inferior vena cava (IVC) and its tributaries are a very rare congenital defect with an incidence of 0.6 to 3% and a wide variety of anatomical variants. In most cases, it is an accidental diagnostic finding in patients undergoing examination for other pathologies. Not infrequently, the anatomic IVC variants are ignored at the stage of exploratory diagnostic assessment due to their rarity, difficulties of identification and perhaps insufficient awareness of a radiologist in this field. It is known that some IVC abnormalities are associated with certain symptoms, and others could be a predictor of deep vein thrombosis. The information on characteristics of the IVC anatomical structure is necessary during interventional manipulation on the retroperitoneal organs and vasculature, because the presence of an atypically located vessel can lead to significant variations in the surgery protocol and potential intraoperative complications. The problems of embryogenesis, classification, variant anatomy, clinical significance and diagnostics of various abnormalities of IVC and its tributaries are discussed in this review.

Imaging evaluation of the inferior vena cava.

The inferior vena cava (IVC) is an essential but often overlooked structure at abdominal imaging. It is associated with a wide variety of congenital and pathologic processes and can be a source of vital information for referring clinicians. Initial evaluation of the IVC is most likely to occur at computed tomography performed for another indication. Many routine abdominal imaging protocols may result in suboptimal evaluation of the IVC; however, techniques to assist in specific evaluation of the IVC can be used. In this article, the authors review the spectrum of IVC variants and pathologic processes and the relevant findings from magnetic resonance imaging, angiography, sonography, and positron emission tomography. Embryologic development of the IVC and examples of congenital IVC variants, such as absence, duplication, left-sided location, azygous or hemiazygous continuation, and web formation, are described. The authors detail IVC involvement in Wilms tumor, leiomyosarcoma, adrenal cortical carcinoma, testicular carcinoma, hepatocellular carcinoma, renal cell carcinoma, and other neoplasms, as well as postsurgical, traumatic, and infectious entities (including filter malposition, mesocaval shunt, and septic thrombophlebitis). The implications of these entities for patient treatment and instances in which specific details should be included in the dictated radiology report are highlighted. Furthermore, the common pitfalls of IVC imaging are discussed. The information provided in this review will allow radiologists to detect and accurately characterize IVC abnormalities to guide clinical decision making and improve patient care.

Budd-Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging.

The goal of this study was to prospectively describe the imaging presentation of hepatic vein (HV) obstruction in patients with Budd-Chiari syndrome (BCS) on duplex and color Doppler ultrasonography (DCD-US), multidetector-row computed tomography (MDCT) and magnetic resonance imaging (MRI). A total of 176 patients with primary BCS (mean age, 33 years; 101 women) were prospectively included. BCS diagnosis was made by direct visualization of HV and/or upper portion of the inferior vena cava (IVC) obstruction on DCD-US and/or MDCT and/or MRI. Location (right, middle, and left HV), type (thrombus, stenosis, or both), and age (recent vs. long-standing) of HV obstruction were described on each imaging examination. HV obstruction was a constant (100%) finding and associated with IVC abnormalities in 51/176 (28.98%) patients. Obstruction of the three HVs was present in 158/176 (89.77%) patients. The prevalences of right, middle, and left HV thrombus were 151/169 (89.35%), 146/169 (86.39%), and 111/169 (65.68%), respectively. Long-standing HV thrombus was observed in more than 92% of patients on the three imaging methods. Agreement between DCD-US, MDCT, and MRI was perfect in the identification of long-standing HV thrombus (κ = 0.9); this agreement was slight to moderate in revealing the type of HV abnormality (i.e., fibrotic cord and non-visible HV). Our results indicate that BCS is a chronic and insidious disease, more often discovered at an advanced stage. These results should warrant further evaluation of screening strategies in patients with risk factors for BCS to identify the disease at an early stage.