Developmental or Procedural Vena Cava Interruption and Venous Thromboembolism: A Review.

The inferior vena cava (IVC) and superior vena cava are the main conduits of the systemic venous circulation into the right atrium. Developmental or procedural interruptions of vena cava might predispose to stasis and deep vein thrombosis (DVT) distal to the anomaly and may impact the subsequent rate of pulmonary embolism (PE). This study aimed to review the various etiologies of developmental or procedural vena cava interruption and their impact on venous thromboembolism. A systematic search was performed in PubMed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines per each clinical question. For management questions with no high-quality evidence and no mutual agreements between authors, Delphi methods were used. IVC agenesis is the most common form of congenital vena cava interruption, is associated with an increased risk of DVT, and should be suspected in young patients with unexpected extensive bilateral DVT. Surgical techniques for vena cava interruption (ligation, clipping, and plication) to prevent PE have been largely abandoned due to short-term procedural risks and long-term complications, although survivors of prior procedures are occasionally encountered. Vena cava filters are now the most commonly used method of procedural interruption, frequently placed in the infrarenal IVC. The most agreed-upon indication for vena cava filters is for patients with acute venous thromboembolism and coexisting contraindications to anticoagulation. Familiarity with different forms of vena cava interruption and their local and systemic adverse effects is important to minimize complications and thrombotic events.

Deep venous thrombosis in patients with atresia of the inferior vena cava and right kidney hypoplasia (KILT syndrome): Systematic review of the literature.

Inferior vena cava (IVC) anomalies are uncommon congenital causes of deep vein thrombosis (DVT). KILT syndrome (kidney and IVC abnormalities with leg thrombosis) has only been described as case reports in the literature. Therefore, the characteristics, evaluation, and management of patients with KILT syndrome have not yet been standardized. This study aimed to systematically review and analyze the clinical and radiographic data and treatment of previously reported cases of KILT syndrome. In this systematic review, we performed a literature search of the PubMed, Scopus, and Web of Science databases in December 2023, with no restrictions on the publication date. After duplicate extractions, 4195 articles were screened. Case reports and case series reporting on KILT syndrome were included. In addition to previously published cases, we included a new case of a previously healthy 25-year-old man with KILT syndrome in the analysis. A total of 34 cases were therefore included in this study. The majority (76.5%) were male patients with a median age of 24 years. In most patients, unprovoked bilateral iliofemoral thrombosis was diagnosed, and 64.7% had left kidney abnormalities. Our study suggests that anomalies of the IVC should be suspected in all young patients, especially male patients, with proximal, recurrent, or idiopathic DVT. If an IVC anomaly is confirmed, the kidneys should be examined to monitor and preserve healthy kidneys in cases of KILT syndrome. The data collected from all patients emphasize the requirement of long-term anticoagulation and risk factor control. Surgical measures may be effective for treating symptomatic refractory cases.

STIC-HD live flow technology in the antenatal diagnosis of scimitar syndrome: A case report.

Scimitar syndrome (SS) is a rare entity with an incidence of approximately 1-3 in 200 000 people. It is typically characterized by complete or partial anomalous pulmonary venous drainage from the right lung into the systemic venous circulation, most commonly the inferior vena cava (IVC). For the first time, we report the diagnosis of SS in a fetus in utero using four-dimensional (4D) spatiotemporal image correlation combined with high-definition live flow rendering mode (STIC-HD live flow).

Atrial fibrillation ablation in a single atrium with inferior vena cava interruption.

Common atrium (CA), also called three-chambered heart, is one of the rare congenital anomalies, defined by a complete absence of the atrial septum, eventually associated with malformation of the atrioventricular (AV) valves. We report the case of a 57-year-old woman with CA complicated with Eisenmenger syndrome and inferior vena cava interruption, who suffered from symptomatic persistent atrial fibrillation (AF). She underwent an initial successful pulmonary vein isolation procedure. A repeat procedure for perivalvular atrial flutter was complicated with inadvertent complete AV block, due to unusual AV node location in this challenging anatomy.

Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review.

BACKGROUND: Nutcracker syndrome (NCS) caused by left renal vein (LRV) entrapment, is one of the most common causes of orthostatic proteinuria. In stereotype, orthostatic proteinuria is often accompanied by left renal vein obstruction and is found in young and underweight individuals. Here, we report a rare case with orthostatic proteinuria in an old obese female caused by a rare type of congenital inferior vena cava (IVC) interruption. CASE PRESENTATION: A 65-year-old obese woman, who suffered from fluctuated proteinuria, had been misdiagnosed as chronic glomerulitis for 30 years. Instead of having any sign of NCS, she had a unique type of IVC interruption. Most venous blood from infrarenal IVC and right kidney drained into her LRV, and then through the expanded communicating vessel, drained into the left ascending lumbar vein which extended as hemiazygos vein. To the best of our knowledge, this is one of the first cases reported of orthostatic proteinuria attributed to the subsequent hemodynamic irregularity caused by IVC interruption without nutcracker phenomenon. CONCLUSION: Adult-onset orthostatic proteinuria is relatively rare, hard to be recognized and could be misdiagnosed as chronic glomerulonephritis. The case provided a novel differential diagnostic condition for those who suffered from fluctuated proteinuria of unknown causes.

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

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

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.

Persistent Left Superior Vena Cava and Inferior Vena Cava Dual Drainage to Coronary Sinus: A Case Report.

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis.

Intra-abdominal haemorrhaging after cardiac catheterisation: the importance of recognising vascular anomalies in heterotaxy syndrome.

A 2-month-old boy with a single ventricle underwent cardiac catheterisation. Inferior vena cava angiography at the end of the examination revealed local stenosis, flexion, and connection to the right hepatic vein. Six hours after catheterisation, he went into haemorrhagic shock. CT revealed contrast extravasation into the liver with ascites. A precise diagnosis of vascular anomalies is mandatory, especially in patients with heterotaxy syndrome.

Duplicated inferior vena cava, the uncommon cause of decreasing extracorporeal membrane oxygenation blood flow: A case report and review.

Duplicated inferior vena cava (DIVC) is a rare anomaly of the venous system, which is mostly found accidentally during intra-abdominal surgery and radiographic study. This anomaly is asymptomatic but may be related to venous thromboembolism in some patients. We present a case of a patient who had inadequate drainage for extracorporeal membrane oxygenation, which was found to be related to duplicated inferior vena cava.

Venous Ulcer in a 5-Year-Old Girl.

BACKGROUND: Agenesis of the inferior vena cava (IVC) is an extremely rare congenital malformation. Although IVC dysplasia can present with symptoms, because of the low prevalence of this disease, it is often omitted from routine examination. Most reports on this topic have described the absence of the IVC; the absence of both a deep venous system and the IVC is even rarer. Chronic venous hypertension and varicosities leading to venous ulcers have been reported in patients with absent IVC that could be surgically bypassed; however, the absence of iliofemoral veins precluded any bypass procedure in the present patient. CASE PRESENTATION: The authors report a case of IVC below renal vein hypoplasia in a 5-year-old girl who presented bilaterally with venous stasis dermatitis and ulcers in the lower extremity limb area. Ultrasonography revealed no clear IVC and iliofemoral venous system under the renal venous plane. Magnetic resonance venography subsequently confirmed the same findings. The patient's ulcers were healed by compression therapy and routine wound care. CONCLUSIONS: This is a rare case of venous ulcer in a pediatric patient stemming from congenital IVC malformation. With this case, the authors demonstrate the etiology of the appearance of venous ulcers in children.

A repeatable CT protocol for quantifying caudal vena cava growth in medium and large breed dogs.

Developmental malformations can cause stunted or abnormal growth and clinical disease in dogs. In humans, measurements of the inferior vena cava are used as methods for detecting abnormal growth trajectories. The objectives of this retrospective, multicenter, analytical, cross-sectional study were to develop a repeatable protocol to measure the caudal vena cava (CVC) and generate growth curves in medium and large-breed dogs during development. Contrast-enhanced CT DICOM images from 438 normal dogs, aged from 1 to 18 months, from five specific breeds were included. A "best guess" measurement protocol was created. Dogs were stratified into medium or large breed groups based on growth rate trajectories. Linear regression models and logarithmic trend lines were used to evaluate the CVC growth over time. The CVC measurements were analyzed from four anatomical regions: thorax, diaphragm, intra-hepatic, and renal. The thoracic segment produced the most repeatable measurements with the highest explanatory power. The CVC thoracic circumference ranged from 2.5 to 4.9 cm from 1 to 18 months of age. Medium and large breeds had similar CVC growth trajectories, with comparable estimated marginal means, however medium dogs reached 80% of predicted final CVC size approximately 4 weeks earlier than large breed dogs. This new protocol provides a standardized technique for evaluation of the CVC circumference over time using contrast-enhanced CT and is most repeatable when taken at the thoracic level. This approach could be adapted for other vessels to predict their growth trajectories, generating healthy reference population data for comparison against patients with vascular anomalies.

Inferior vena cava thrombosis-rope ladder sign.

Congenital heart disease comprises one of the largest groups of congenital defects, affecting approximately 1% of births. Advances in pre- and postoperative critical care treatment as well as surgery and interventional procedures have improved survival rates, but treatment and long-term care of children with complex congenital heart disease remains challenging, and is associated with a number of complications.Here, we report on a 17-month-old infant with congenital univentricular heart disease who devloped post-operatively inferior vena cava (IVC) thrombosis. IVC thrombosis was confirmed by a bedside contrast media study (X-ray) demonstrating collateral paravertebral circulation along the paravertebral sinuses bilaterally into the azygos and hemiazygos vein ("rope ladder sign"), with no contrast media detected in the IVC. The infant was subsequently started on aspirin and clopidogrel.

RESULTS OF TREATMENT OF DEEP VEINS THROMBOSIS IN PATIENTS WITH CONGENITAL ANOMALIES OF THE INFERIOR VENA CAVA.

Features of treatment of venous thromboembolism in patients with abnormalities in the development of the inferior vena cava. Congenital anomalies of IVC development are a rather rare pathology. The analysis of treatment results will better understand the pathophysiology of the disease and form an optimal treatment strategy. We analyzed the results of treatment of 1243 patients with venous thromboembolism from 2010 to 2022. Congenital anomalies of inferior vena cava(IVC) development were diagnosed in 2 (0.16%) patients. One patient had IVC aplasia, the other had IVC hypoplasia. Both patients had bilateral iliofemoral deep vein thrombosis(DVT). For the treatment of patients, we used anticoagulant therapy and elastic compression of the lower extremities. During the study period, venous thromboembolic complications in the form of pulmonary embolism were not recorded, fatal complications were not diagnosed. Thus, the results of treatment of patients with venous thromboembolism and anomalies in the development of IVC suggest that the use of anticoagulant therapy in this category of patients allows to obtain positive results of treatment.

Systematic review of electrophysiology procedures in patients with obstruction of the inferior vena cava.

AIMS: The objective of the study was to conduct a systematic review to describe and compare the different approaches for performing cardiac electrophysiology (EP) procedures in patients with interrupted inferior vena cava (IVC) or equivalent entities causing IVC obstruction. METHODS: We conducted a structured search to identify manuscripts reporting EP procedures with interrupted IVC or IVC obstruction of any aetiology published up until August 2020. No restrictions were applied in the search strategy. We also included seven local cases that met inclusion criteria. RESULTS: The analysis included 142 patients (mean age 48.9 years; 48% female) undergoing 143 procedures. Obstruction of the IVC was not known before the index procedure in 54% of patients. Congenital interruption of IVC was the most frequent cause (80%); and, associated congenital heart disease (CHD) was observed in 43% of patients in this setting. The superior approach for ablation was the most frequently used strategy (52%), followed by inferior approach via the azygos or hemiazygos vein (24%), transhepatic approach (14%), and retroaortic approach (10%). Electroanatomical mapping (58%), use of long sheaths (41%), intracardiac echocardiography (19%), transesophageal echocardiography (15%) and remote controlled magnetic navigation (13%) were used as adjuncts to aid performance. Ablation was successful in 135 of 140 procedures in which outcomes were reported. Major complications were only reported in patients undergoing AF ablation, including two patients with pericardial effusion, one of whom required surgical repair, and another patient who died after inadvertent entry into an undiagnosed atrioesophageal fistula from a previous procedure. CONCLUSION: The superior approach is most frequent approach for performing EP procedures in the setting of obstructed IVC. Transhepatic approach is a feasible alternative, and may provide a "familiar approach" for transseptal access when it is required. Adjunctive use of long sheaths, intravascular echocardiography, electro-anatomical mapping and remote magnetic navigation may be helpful, especially if there is associated complex CHD. With careful planning, EP procedures can usually be successfully performed with a low risk of complications.

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

OBJECTIVE: To evaluate the prevalence of inferior vena cava (IVC) anomalies in an asymptomatic healthy population and symptomatic patients. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

An unexpected route of temporary pacing lead.

Congenital anomalies of the venous system including superior (SVC) and inferior vena cava are not uncommon. Failure of certain vessels embryogenesis results in numerous caval variations and anomalies. Anomalies of systemic venous circulation are frequently seen in patients with congenital heart disease and are sometimes incidentally recognized. However, these are usually without significant clinical implications, detection of these anomalies is necessary to avoid diagnostic pitfalls and for planning vascular interventions. We present a rare caval anomaly, a left-sided IVC with hemiazygos continuation to left SVC and coronary sinus.

Anomalies of Inferior Vena Cava: Implications and Considerations in Retroperitoneal Surgical Procedures.

Anomalies of the inferior vena cava pose a great challenge to surgeons. Although uncommon, these congenital vascular malformations may have significant surgical implications. Awareness of their presence is essential to avoid inadvertent injury and major bleeding during retroperitoneal procedures. An accurate preoperative diagnosis and detailed planning play a crucial role to obtain successful outcomes when confronted with them. Several surgical techniques have been recommended to protect these anomalous venous structures. The aim of this review is to contribute to the knowledge of the most common types of anomalies of inferior vena cava encountered during retroperitoneal procedures. With this purpose, a summary of their anatomy, embryology, incidence, diagnosis and intra-operative management is presented.

Disruption of the glomerular basement membrane associated with nutcracker syndrome and double inferior vena cava in Noonan syndrome: a case report.

BACKGROUND: Nutcracker syndrome (NCS) is characterized by compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery. While rare, NCS was reported to be accompanied by double inferior vena cava (IVC). We herein report a case of Noonan syndrome (NS) with double IVC who presented with macrohematuria and proteinuria. CASE PRESENTATION: The patient was a 23-year-old man, who had been diagnosed with NS due to RIT1 mutation, after showing foamy macrohematuria 3 weeks previously. A physical examination revealed low-set ears and a webbed neck. A urinalysis showed hematuria and proteinuria, and urinary sediments showed more than 100 isomorphic red blood cells per high-power field. His proteinuria and albuminuria concentrations were 7.1 and 4.5 g/g⋅Cr, respectively. Three-dimensional contrast-enhanced computed tomography (CT) showed double IVC and narrowing of the LRV after interflow of the left IVC. The aortomesenteric angle on a sagittal reconstruction of the CT image was 14.7°. Cystoscopy revealed a flow of macrohematuria from the left ureteral opening. On Doppler ultrasonography, there was scant evidence to raise the suspicion of the nutcracker phenomenon. Since severe albuminuria continued, a left kidney biopsy was performed. Light microscopy showed red blood cells in Bowman's space and the tubular lumen. Electron microscopy revealed disruption of the glomerular basement membrane (GBM). Vulnerability of the GBM was suspected and a genetic analysis revealed a heterozygous mutation at c.4793 T > G (p.L1598R) in the COL4A3 gene. Screening for coagulation disorders revealed the factor VIII and von Willebrand factor (vWF) values were low, at 47.6 and 23%, respectively. A multimer analysis of vWF showed a normal multimer pattern and he was diagnosed with von Willebrand disease type 1. As the bleeding tendency was mild, replacement of factor VIII was not performed. His macrohematuria and proteinuria improved gradually without treatment, and his urinalysis results have been normal for more than 6 months. CONCLUSIONS: The present case showed macrohematuria and proteinuria due to NCS in NS with double IVC and von Willebrand disease type 1. The macrohematuria and proteinuria originated from glomerular hemorrhage because of vulnerability of the GBM due to COL4A3 mutation.

The role of the hepatic factor on the improvement of arterial oxygenation after a Kawashima procedure: A case report.

BACKGROUND: Anomalies of the systemic venous return to the heart are infrequent, occurring more often in patients with congenital heart disease. CASE REPORT: We present a patient with heterotaxy, univentricular circulation, and hemiazygos return who underwent a Kawashima procedure at 3 years of age. Fourteen years later, she developed increased cyanosis and tiredness. The symptoms clearly improved after an off-pump derivation of the hepatic drainage from the common atrium to the hemiazygos system. CONCLUSIONS: This rare case report addresses the surgical challenges imposed by patients with complex congenital heart disease combined with anomalies in systemic venous return. Although improvement in arterial oxygenation after redirecting hepatic flow to the hemiazygos system in patients previously submitted to a Kawashima procedure has already been reported, the existence of a hepatic factor remains to be recognized.