Absence Of Inferior Vena Cava Research Articles

Hepatic-to-azygos vein redirection after a failed bifurcated graft Fontan conversion

A successful hepatic-to-azygos vein redirection was performed in a patient with absent inferior vena cava using a long vascular graft to address a pulmonary arterio-venous fistula after a failed Fontan conversion. No exacerbation was observed 5 years postoperatively.

Difficulty in the Diagnosis of Biliary Atresia Splenic Malformation Syndrome In Utero.

The fetus of a 30-year-old pregnant Japanese woman was diagnosed with absence of inferior vena cava (IVC) and azygos continuation of interrupted IVC without cardiac anomalies at 34 weeks of gestation, and a healthy male neonate weighing 2,910 g was delivered at 37 weeks of gestation. On day 42 after birth, direct bilirubin predominant hyperbilirubinemia and high serum gamma-GTP levels were detected. Computed tomography revealed the presence of a lobulated and accessory spleen, and laparotomy demonstrated type III biliary atresia (BA), confirming the final diagnosis of BA splenic malformation (BASM) syndrome. In retrospect, non-visualization of the gallbladder was missed in utero. The combination of the absence of IVC and BA without cardiac anomalies is far less likely to occur in left isomerism. Although BA remains difficult to detect in utero, special attention should be paid to cases of BA associated with findings of left isomerism, including the absence of IVC, to enable early diagnosis and treatment of BASM.

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.

POLIESPLENIA E ANOMALIA DO SISTEMA PORTAL HEPÁTICO ASSOCIADAS À AUSÊNCIA DE VEIA CAVA INFERIOR EM CRIANÇA: RELATO DE CASO

Agenesis of inferior vena cava (IVC)is a rare cardiovascular malformation that occurs between the sixth and tenth week of embryogenesis. It may be associated with cardiac and abdominal malformations, besidescomplications such as deep vein thrombosis (DVT). The present study reports the case of a patient randomly diagnosed with total IVC agenesis associated with malformation of the portohepatic system and polysplenia. Female patient, 9 years old, being monitored for hemorrhagic dengue, withcomputed tomographyand abdominal ultrasound revealing anomaly of the development of the hepatic portal system associated with the absence of inferior vena cava with continuation inretrocrural azygos vein, in addition to multiple accessory spleens. When the anastomoses of the IVCprimitive veins are not formed properly, partial or total agenesis of this vessel may occur. Consequently, the blood is diverted to the retrocrural azygos. Identification of these malformations is important for the patients receive proper guidance about DVT prevention, in additionto monitoring them for diagnosis of other possible malformations.

Infrarenal inferior vena cava agenesis

We introduce the case of a 28-year-old male patient, who was admitted to the Vascular Department with a diagnosis of infrarenal inferior vena cava agenesis and secondary deep vein thrombosis (DVT) in the lower limbs (LL). A 28-year-old male patient who came to the hospital’s Emergency Department due to non-specific abdominal discomfort described as "bloated feeling", dull pain in the mesogastrium and oedema in the LL during a month, for which he had consulted his primary care facility on several occasions. Upon arrival at the Emergency Department, the physical examination revealed mild bilateral bimalleolar oedema, with no other significant findings. A complete blood test was performed, showing a D-dimer of 5,600 IU/l. In view of the suspicion of DVT in the LL, a Doppler echocardiography was performed, which reported filling defects inside the common femoral vein and both bilateral external and internal iliac veins. Due to these findings, he was admitted to the Vascular Department to complete the study. During admission, an abdominal/pelvic CT scan was performed, showing the following results: absence of inferior vena cava at the infrarenal level, large amount of collateral circulation – especially paravertebral lumbar, multiple pelvic varicose veins and filling defects in the common bilateral external and internal iliac veins (Figure 1). Given the aforementioned findings, treatment with low-molecular-weight heparins and subsequent anticoagulation therapy was started after hospital discharge.

An Extremely Rare Case of Intestinal Malrotation with an Absent Inferior Vena Cava.

We present a case of intestinal malrotation with an absent inferior vena cava, which was found in a cadaver during a dissection course in our medical school. The intestinal malrotation was Amir-Jahed type 2, with the large intestine on the right side and the small intestine on the left side of the abdominal cavity. The descending colon was fixed on the right side of the posterior abdominal wall and continued into the pelvic cavity from the right side. The cadaver also had a venous system anomaly. The pre-renal segment of the inferior vena cava, which is a section between the renal vein and the hepatic vein, was absent. The inferior vena cava connected to the azygos vein after being joined by bilateral renal veins. The only hepatic segment of the inferior vena cava, which was posterior to the liver and received hepatic veins, penetrated the diaphragm and flowed into the right atrium. To our knowledge, this is the first report of these two anomalies appearing concurrently. We discuss the details of this case and the embryological considerations.

Unusual long survival in a case of heterotaxy and polysplenia

Heterotaxy syndrome with polysplenia is an extremely rare congenital disorder caused by a disruption in the embryonic development that results in an abnormal arrangement of the abdominal and thoracic organs. We present the case of a 59-year-old female patient with invasive ductal carcinoma of the right breast (luminal A type) and CT findings of heterotaxy syndrome with polysplenia. The most remarkable anomalies identified were a left inferior vena cava draining into the hemiazygos vein, absent inferior vena cava at the thoracic level, and hepatic veins directly draining into the right atrium. Moreover, an atrial septal defect was identified, explaining the pulmonary hypertension of unknown cause previously detected in the patient. The relevance of this case lies in the unusual anatomical abnormalities found and the large patient survival, having in to account the great rate of heterotaxy syndrome mortality in the first years of life.

Non-standard hepatic venous reconstruction in the setting of absent inferior vena cava in partial graft pediatric liver transplantation-A matched retrospective cohort study.

Recipient cava may be unavailable for outflow reconstruction in some children undergoing liver transplantation (PLT) due to caval agenesis, tumor, or fibrotic caval occlusion. Non-standard hepatic venous reconstruction (NHVR) with a direct veno-caval anastomosis or neo-cava reconstruction is necessary in such cases. Retrospective review of all PLT needing NHVR performed in our unit from January 2010 to September 2019 was performed. Outcomes of this group were compared to a 2:1 matched control group who underwent transplantation with standard piggyback technique. Fifteen children (4.9%) of 304 PLT recipients underwent NHVR. Caval agenesis in biliary atresia (n=5, 33%) and hepatoblastoma infiltrating the cava (n=4, 27%) were the commonest indications. Ten children had neo-cava reconstruction, while 5 had direct anastomosis to the supra-hepatic caval cuff or right atrium. One child had developed neo-cava thrombosis without graft venous outflow obstruction in the post-operative period. There was no significant difference in major morbidity, need for re-operation (20% vs 16.7%; P=1.00), hospital stay (24days, vs 21days; P=.32), graft & patient survival among the study and control groups. Absent or inadequate recipient cava during PLT with a partial liver graft can be safely managed with technical modifications. Results equivalent to standard piggyback implantation can be achieved.

Oxy‐RVAD support for lung transplant in the absence of inferior vena cava

Cardiopulmonary bypass and extracorporeal membrane oxygenation are commonly used adjuncts to lung transplantation. These techniques are not without associated morbidity and mortality, and the surgeon must be aware of the possibility of aberrant anatomy that could lead to vascular injury during cannulation. In this report, we describe a patient with congenital absence of the inferior vena cava undergoing lung transplantation who required perioperative cardiopulmonary support. A percutaneous dual lumen cannula, Protek Duo, was connected in an Oxy-RVAD configuration to provide right ventricular and oxygenation support both intraoperatively and postoperatively to this patient.

Hybrid Radiofrequency Ablation for a Patient with WPW Syndrome and Complicated Cardiovascular Malformation

Wolff-Parkinson-White (WPW) syndrome causes paroxysmal supraventricular tachycardia (PSVT) and catheter ablation is the most effective therapy method. We summarized a rare case of WPW syndrome. The patient suffered from WPW syndrome complicated with an atrial septal defect (ASD), persistent left superior vena cava, and absence of inferior vena cava. A hybrid operation was arranged to solve his problem. During the procedure, we did endocardial and epicardial mapping and ablation, respectively. Conduction of the accessory pathway could be temporarily obstructed, but recovered soon later. Considering it was a rare and interesting case worth recording and discussing, we share it with the public.

SAT-167 THE CASE OF A NOT SO ABSENT INFERIOR VENA CAVA

Background: Congenital absence of the inferior vena cava (IVC) is an extremely rare condition, which may be congenital or due to perinatal thrombosis, is associated with lower limb deep vein thrombosis risk, non-healing venous ulcers and the development of collateral venous drainage systems. We report a case of a congenitally absent IVC in a patient with renal failure and subsequent renal transplantation, with recurrent acute kidney injury (AKI) related to exercise, whom underwent successful IVC stenting with restoration of venous flow. Case: The patient was a 28-year-old female with a diagnosis of congenital absence of her IVC, with venous drainage of her lower limbs achieved by markedly dilated ascending lumbar veins which drained into an enlarged azygous vein. She had a history of chronic kidney disease with renal transplant at age 4, which failed after recurrent unexplained episodes of acute tubular necrosis (ATN). After her second transplant at age 26 she continued to have recurrent episodes of biopsy proven ATN, which were subsequently found to be related to exercise with creatinine levels of more than 300µmol/L from a baseline of 113µmol/L. Pelvic angiography demonstrated abrupt pinching of the enlarged right lumbar vein at the L4/L5 vertebrae with straight leg raise and subsequent flow of blood into the left lumbar vein. The dilated ascending lumbar veins in the retroperitoneum, which drain the renal transplant, course between the psoas muscle and the lumbar vertebral bodies. During exercise, it is hypothesised that there is venous hypertension in the kidney due to outflow obstruction from compression of the ascending lumbar veins (from psoas muscle compression) at the level of L4/L5. There is subsequent decreased venous return, leading to arterial hypotension, poor renal perfusion, and AKI. These episodes recovered with intravenous fluids and bed rest. Botox injections into the psoas muscles were minimally beneficial in reducing exercise related AKI and complicated by a rash. CT venogram identified suggestion of a chronically occluded IVC remnant, with contrast present in the native kidneys, which appeared small and unscarred, as well as a thin (2mm) cord extending from the liver to the transplant kidney (See Image 1). Subsequently, she underwent endovascular balloon recanalization of the right common iliac vein and IVC to 14mm with restoration of antegrade flow. Subsequent venogram demonstrated some elastic recoil and collapse of the recanalized IVC, so she underwent dilatation of the IVC to 18mm with iliocaval segment reconstruction with stents (20mm caval stents and 10mm iliac stents) shown in Image 2. Repeat imaging 2 days later demonstrated patent stenting in the common iliac vein and IVC, with persisting dilated azygous system, however the lumbar veins had decreased in calibre. Post stenting, her creatinine was 115µmol/L increasing to 144µmol/L after walking 20,000 steps/day for two consecutive days. Image 1: CT venogram showing chronically occluded IVC remnant (indicated with arrow). Image 2: Angiography showing successful IVC stent placement. ▪▪▪ ▪▪▪ The aim of iliocaval reconstruction was to provide a low resistance pathway for venous drainage which would not be subject to extrinsic compression. Iliocaval reconstruction appears to have improved the effect of exercise on renal function.

Missing inferior vena cava: report of a rare case

Missing inferior vena cava (IVC) is an uncommon but well recognised anomaly. Our case was a 32 year old male, presented to us with features suggestive of gastric outlet obstruction. When evaluated he was found to have gastric outlet obstruction secondary to cicatrized duodenum and a large subcutaneous vein running from supra pubic region towards the left axilla. Further imaging revealed the complete absence of inferior vena cava and the large collateral drainng the venous blood from lower half of the body into left interal jugular vein. Surgical correction of this rare anomaly is not required in the absence of deep vein thrombosis and venous ulcers of lower extremity. Here we report a case of young male with incidentally detected missing IVC as a contribution to the limited literature available on this rare anomaly.

Diagnosis and prognosis of fetal inferior vena cava malformation by fetal echocardiography

Objective To summarize the characteristics of two-dimensional(2D) ultrasound, spatiotemporal imaging correlation(STIC) and vascular casting of fetal inferior vena cava(IVC) malformations, analyze the clinical characteristics and the prognosis of fetal IVC malformations, and explore the early diagnostic value of fetal echocardiography for fetal IVC malformations. Methods Sixty-one cases of fetal IVC malformation diagnosed by fetal echocardiography in Beijing Anzhen Hospital Maternal Fetal Medical Consultation Center were retrospectively analyzed. The clinical data, echocardiographic features, genetic testing information and prognosis were summarized. Results ①In the 61 cases of IVC malformation, there were 51 cases of absence of IVC, 46 cases of absence of liver segment, 5 cases of absence of left IVC hepatic segment, 9 cases of left IVC (including 5 cases of left IVC and absence of IVC hepatic segment), 5 cases of double IVC, and 1 case of right hepatic vein reflux. ②Ten fetuses of the 61 fetuses were simple IVC deformity without any other malformations. Other venous malformations included 7 cases of absence of right superior vena cava and persistent left superior venacava, 2 cases of absence of venous catheters, and 3 cases of persistent right umbilical vein, 2 cases were merged alone; 49 cases were merged with intracardiac structural malformations and arrhythmia. ③Thirty-eight pregnant women underwent Down′s screening or non-invasive DNA, all of which were at low risk.Four cases underwent amniocentesis, and no obviously abnormality was found. Sixteen cases underwent autopsy and genome sequencing after induced labor, one was single gene abnormalities (ZFPM2 classical mutation). ④STIC images were collected in 20 cases, and 19 cases were clearly visualized. ⑤Vascular cast was made to show the shape of blood vessel more clearly and stereoscopically in 4 cases of absent hepatic segment of IVC. ⑥Eleven fetuses were born and confirmed from the results of postnatal ultrasound, who were healthy in follow-up; 16 fetuses were induced labor; 33 fetuses were losed during follow-up; 1 fetus died after birth (arrhythmia). Conclusions Fetal echocardiography combined with STIC technology can clearly diagnose the anomalies of IVC. Vascular casting can provide stereoscopic and visual manifestations of the anomalies of IVC. The prognosis of simple anomalies of IVC is good. Key words: Echocardiography, Fetal; Inferior vena cava malformation; Spatiotemporal imaging correlation; Vascular cast

Absent inferior venacava and anti phospholipid antibody syndrome: Compounding risk factor for deep vein thrombosis and recurrent pregnancy loss

The spectrum of congenital anomalies of the inferior vena cava (IVC) has been well described in the literature, but the absence of IVC is a rare occurrence. Most of these patients remain asymptomatic, and the age of presentation depends on the development of deep-venous thrombosis. A young female presented to us with irregular bleeding per vaginum and recurrent pregnancy loss. A thorough general examination of the female patient revealed dilated veins over the abdomen and lower limbs. Investigations of recurrent pregnancy loss led to the diagnosis of antiphospholipid antibody syndrome and computed tomography venography to find out the cause of dilated veins lead to the diagnosis of absent IVC. Both these factors, i.e., absent IVC and the presence of autoimmune antibodies, have compounded the risk factor of thrombosis and management challenging which is discussed.

Technical considerations in liver transplantation for biliary atresia with situs inversus: A single center experience and a literature review

Introduction: In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. The surgical procedures and the outcomes in our patients and reviewed previously-reported cases undergoing LT for BA with SI were evaluated. Method: Between November 2005 and October 2018, 235 children underwent LT with an overall 10-year graft survival of 94.7%. Of these, 6 patients (2.6%) received LT for BA with SI. Result: Living donor LT (LDLT) was applied in all the patients. Vascular anomalies included the absence of the inferior vena cava (IVC) (n=5), preduodenal portal vein (PV) (n=4), and aberrant hepatic artery (HA) (n=1). Hepatic vein (HV) outflow obstruction occurred in one patient with absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in one patient with preduodenal PV five months after LDLT. Overall patient survival was 80% at 3 years. Forty-nine cases of LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction in 2.0%, PV complications in 10.2%, and HA thrombosis in 4.1% of the cases. All PV complications occurred in patients with preduodenal PV (5/23; 21.7%) undergoing LDLT. Conclusion: The assessment of the details of the surgical procedures and the information of systemic review demonstrated excellent outcomes of LT for BA with SI, and the incidence of complications associated with vessel anomalies except preduodenal PV was unremarkable.

EP04.21: Tetralogy of Fallot: a series of cases – associations and evolution

Tetralogy of Fallot has many forms and is frequently associated with other fetal malformations. The evaluation of this pathology's variants, the structural anomalies associations and the fetal or neonatal evolution are observed on a series of cases. A series of 18 fetuses were diagnosed with tetralogy of Fallot, through a period of 7 years, in a tertiary centre from Romania. The gestational age at the time of the echocardiography ranged between 21 and 38 weeks. An amniocentesis was performed in 7 fetuses, out of which one had Down syndrome, two had DiGeorge syndrome, while the remaining four fetuses had a normal karyotype. In three of the fetuses, the tetralogy of Fallot had an extreme variant: one case had a pulmonary valve atresia with severe hypoplasia of the pulmonary branches and MAPCA's, the other was diagnosed with severe pulmonary valve stenosis and pulmonary branch hypoplasia (DiGeorge syndrome) and one fetus had an absent pulmonary valve. Other structural anomalies associations were described in 8 fetuses: bilateral pyelectasis in two of the cases (one with Down, the other with an absent inferior vena cava), hypoplastic thymus in two of the fetuses (with Down and DiGeorge syndrome), unique kidney in one fetus (absence of the pulmonary valve), one fetus with early IUGR and unique umbilical artery (normal karyotype), one case with polycystic bilateral kidney and 2 cases with LPSVC (one of them with MAPCA's). Out of the 18 fetuses, four underwent an TOP (the cases with Down, early IUGR, bilateral polycystic kidney and absence of the pulmonary valve). A number of 7 pregnancies came back to our clinic, and the 7 newborns underwent a cardiac evaluation, followed by surgical interventions which included a systemic-pulmonary shunt. An additional MAPCA's was diagnosed after birth. In conclusion the associations between tetralogy of Fallot and other structural anomalies are frequent, and the MAPCA's diagnosis can't always be intrauterinally established. This research was partially supported by the Collective Research Grants of the University of Medicine and Pharmacy Tîrgu Mureş, Romania (“The role of mother's genetic determinism in child's obesity correlated with bioimpedance and anthropometric parameters” no.275/4/11.01.2017).

Absence of Inferior Vena Cava in a Renal Transplant Recipient: A Case Report

A 27-year-old woman was admitted to our department with end-stage renal failure due to reflux nephropathy. She had no history of deep venous thrombosis. After pretransplantation evaluation, her father was accepted for kidney donation. We observed intraoperatively that the patient's iliac veins and inferior vena cava (IVC) were absent. There were many venous collaterals, but none of them was dilated enough for renal vein anastomosis. Since we could not find a suitable vein for venous drainage of the allograft, we decided to stop donor surgery and postpone renal transplantation (RT) for detailed radiologic examination. Contrast-enhanced computed tomography revealed the absence of an infrahepatic segment of IVC. Superior mesenteric vein was thin. Portal and splenic veins were normal, but we decided not to use them for venous drainage because of increased risk of torsion. We informed the patient and her family about the situation and cancelled RT. Iliac vein and IVC anomalies are not absolute contraindications for RT, but when a dilated collateral vein is not present or when there is no option for safe renal vein anastomosis as in our case, RT may not be possible.

Absent Inferior Vena Cava with Recurrent Deep Venous Thrombosis

Introduction: A congenitally absent inferior vena cava (IVC) is a rare condition that is recognized to be associated with deep venous thrombosis (DVT), especially in young adults. It may be not apparent until later in life.

Prenatal diagnosis of isolated interrupted inferior vena cava with azygos continuation to superior vena cava

Absence of inferior vena cava is an uncommon congenital abnormality. It is usually associated with other structural anomalies, typically left isomerism. We report a case of interrupted inferior vena cava with azygos continuation diagnosed as an isolated finding during routine prenatal ultrasound scan, confirmed by post-natal echocardiography. Detailed ultrasound examination of the fetal anatomy failed to demonstrate other anomalies. The neonatal course of this fetus was uneventful.

Heterotaxy syndrome associated with left ventricular non-compaction

A 20-year-old asymptomatic man was referred to our clinic for the evaluation of a 2/6 grade systolic cardiac murmur. The ECG was normal. On his transthoracic echocardiography (TTE), subaortic membrane causing peak 25 mm Hg gradient on left ventricular outflow tract as the cause of the murmur (Fig. 1A) and an enlarged coronary sinus (CS) (Fig. 1B) were detected. We also suspected left ventricular non-compaction (Fig. 1C, D). We performed agitated saline injection via left antecubital vein for diagnosis of persistent left superior vena cava (PLSVC) and bubbles reached to CS before right atrium, indicating PLSVC. The injection was repeated via right antecubital vein to evaluate the right superior vena cava (RSVC) because of that the absence of RSVC may be associated with PLSVC. After the injection, the coronary sinus was opacified before right atrium, indicating the absence of RSVC. Left ventricular non-compaction was confirmed with cardiac magnetic resonance imaging (Fig. 2A, B). Thoracal computed tomographic (CT) venography was performed, and it confirmed the PLSVC and also revealed the absence of RSVC (Fig. 3A). Surprisingly, it also demonstrated absent inferior vena cava (IVC). Therefore, it forced us to perform abdominal CT angiography. It revealed the venous return of lower limbs and abdomen maintaining with dilated hemiazygous vein, indicating absent IVC. His abdominal CT faced us to another interesting findings, polysplenia (Fig. 3B). Subsequently, all findings in the entire story reached us to diagnose heterotaxy syndrome (HS). Coexistence of HS, subaortic membrane and left ventricular non-compaction has not been reported in the literature.