Fracture of inferior vena cava stent after endovascular treatment for Budd-Chiari syndrome:A case series and literature review

Budd-Chiari syndrome(BCS)is a rare condition characterized by hepatic venous outflow obstruction.Balloon angioplasty,with or without stenting,is the recommended first-line treatment modality in Asian countries.As a supplement to balloon angioplasty,expandable metallic Z-stent deployment can effectively improve long-term inferior vena cava(IVC)patency.Although stent placement is a standard and frequently performed treatment,very few IVC stent-related complications,such as stent fractures,have been reported.Here we present a case series and a comprehensive review of IVC stent fractures in patients with BCS.The most common characteristic of IVC stent fractures is a protrusion of the proximal segment of the IVC stent into the right atrium and its systolic and diastolic movements along with heart rhythms.Accurate stent deployment,large-diameter balloon dilation,patient breath-holding training,preferential selection of a triple stent,and the use of an internal jugular vein approach to stent deployment may ensure precise stent localization and avoid postoperative complications.

A case of duplicated inferior vena cava with bilateral iliac vein compression

Duplicated inferior vena cava with bilateral iliac vein compression is extremely rare.We report a case of an 87-year-old man presented with bilateral lower extremity swelling,who was noted to have duplicated inferior vena cava,as revealed by computed tomography angiography(CTA).This revealed bilateral iliac vein compression caused by surrounding structures.Anticoagulant treatment combined with stent implantation completely alleviated this chronic debilitating condition during the follow-up of 2 months with no recurrence.

Complex inferior vena cava reconstruction during ex vivo liver resection and autotransplantation:A case report

BACKGROUND Ex vivo liver resection and autotransplantation(ELRA)is an essential approach for treating patients with end-stage hepatic alveolar echinococcosis(AE),and its surgical indications involve severe invasion of important hepatic vessels,which makes in vivo resection impossible.Revascularization is a major step in the process of ELRA,which is extremely challenging when the invaded vessels have huge defects.CASE SUMMARY Herein,we have reported the case of a 26-year-old patient with hepatic AE in an autologous liver graft who underwent complex inferior vena cava(IVC)reconstruction using disease-free IVC,autologous portal vein fragments,and umbilical vein within the ligamentum teres hepatis.The patient showed good surgical recovery without vascular-related complications during the long-term follow-up.CONCLUSION We reviewed three studies that have reported complex revascularization of the IVC.This case report and systematic review showed that the use of autologous perihepatic vessels prevents donor-area trauma,immune rejection,and other adverse reactions.When the blood vessel is severely invaded and a single vascular material cannot repair and reconstruct the defect,ELRA may provide a safe and feasible surgical approach,which has good prospects for clinical application.

Surgical treatment of hepatocellular carcinoma with inferior vena cava tumor thrombus: a new classification for surgical guidance

BACKGROUND: Hepatic resection is the main treatment modality for hepatic tumors. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The present study aimed to evaluate hepatectomy and resection of inferior vena cava tumor thrombus (IVCTT) in patients with hepatocellular carcinoma, and the relationship between IVCTT classification and selection of surgical technique. METHODS: We retrospectively reviewed 13 patients with hepatocellular carcinoma who had undergone hepatectomy with IVCTT resection between May 1997 and August 2009. Age, gender, diagnosis, findings of physical examination, results of preoperative laboratory investigations, radiological examination, criteria for resection, postoperative pathological results, incisions, operative technique, intraoperative transfusion, drains, and intraoperative and postoperative complications were evaluated for all patients. RESULTS: Type Ⅰ IVCTT (10 patients) was posterior to the liver and below the diaphragm; type Ⅱ IVCTT (2 patients) was above the diaphragm but still outside the atrium; and type Ⅲ IVCTT (1 patient) was above the diaphragm and in the right atrium. Type Ⅰ was treated by radical hepatectomy and removal of IVCTT with total hepatic vascular exclusion. Type Ⅱ was treated by radical hepatectomy and removal of IVCTT by incision of the diaphragm. Type Ⅲ was treated by hepatectomy and resection of the thrombus from the right atrium under cardiopulmonary bypass. There were no surgical complications and one patient has been survived for 4 years with cancer-free status. The median survival time was 18.2 months, and the 1-and 2-year survival rates were 53.8% and 15.4%, respectively. CONCLUSION: Surgical treatment is safe and feasible for treatment of IVCTT in patients with hepatocellular carcinoma, and surgical resectability can be judged according to the classification of tumor thrombus.

Klippel-Trenaunay syndrome with gastrointestinal bleeding,splenic hemangiomas and left inferior vena cava

Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins,cutaneous capillary malformation,and hypertrophy of bone and(or)soft tissue.Gastrointestinal vascular malformations in Klippel-Trenaunay syndrome may present with gastrointestinal bleeding.The majority of patients with spleenic hemangiomatosis and/or left inferior vena cava are asymptomatic.We herein report a case admitted to the gastroenterology clinic with life-threatening hematochezia and symptomatic iron deficiency anemia.Due to the asymptomatic mild intermittent hematochezia,splenic hemangiomas and left inferior vena cava,the patient did not seek any help for gastrointestinal bleeding until his admittance to our department for evaluation of massive gastrointestinal bleeding.He was referred to angiography because of his serious pathogenetic condition and inefficiency of medical therapy.The method showed that hemostasis was successfully achieved in the hemorrhage site by embolism of corresponding vessels.Further endoscopy revealed vascular malformations starting from the stomach to the descending colon.On the other hand,computed tomography revealed splenic hemangiomas and left inferior vena cava.To the best of our knowledge,this is the first Klippel-Trenaunay syndrome case presenting with gastrointestinal bleeding,splenic hemangiomas and left inferior vena cava.The literature on the evaluation and management of this case is reviewed.

Inferior vena cava obstruction and collateral circulation as unusual manifestations of hepatobiliary cystadenocarcinoma

BACKGROUND: Hepatobiliary cystadenocarcinoma represents a rare epithelial malignant tumor derived from the intrahepatic bile duct. METHODS: A 71-year-old woman, who had undergone laparoscopic drainage of a cystic lesion of the right hepatic lobe, was misdiagnosed as having hepatic echinococcal disease, and received intracystic infusion of 95% ethanol four years ago. She was admitted to our hospital for further treatment. RESULTS: Physical examination revealed dilated superficial veins across the right abdominal wall. After mapping the direction of blood flow in these vessels, we assumed that this was a sign of inferior vena cava obstruction. Abdominal ultrasound, computed tomography, magnetic resonance imaging combined with magnetic resonance angiography showed a large cystic mass in the right upper quadrant and epigastrium, displacing the adjacent structures, adherent to the inferior vena cava, which was not patent, resulting in dilation of superficial epigastric veins. The patient underwent an exploratory laparotomy. Total excision of the huge mass measuring 16×15 cm was possible under selective vascular exclusion of the liver. Removal of the tumor resulted in immediate restoration of flow in the inferior vena cava. On the basis of the pathology and findings of immunohistochemical analysis, a hepatobiliary cystadenocarcinoma was diagnosed.CONCLUSIONS: In the present case, hepatobiliary cystadenocar-cinoma was accompanied by dilated superficial venous collaterals due to inferior vena cava obstruction. Selective vascular exclusion of the liver allowed a safe oncological resection of the tumor.

Liver cirrhosis in hepatic vena cava syndrome(or membranous obstruction of inferior vena cava)

Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.

Sclerosing epithelioid fibrosarcoma of the liver infiltrating the inferior vena cava

Sclerosing epithelioid fibrosarcoma （SEF） is a rare and distinct variant of fibrosarcoma, composed of epithelioid tumor cells arranged in strands, nests, cords, or sheets embedded within a sclerotic collagenous matrix. We report a 39-year-old man with SEF of the liver, which infiltrated the inferior vena cava （IVC）. The SEF of the liver was successfully resected, and the infiltrated IVC was also removed together with the liver tumor. Histopathological examination of the tumor showed typical histopathology of SEE Immunohistochemically, the tumor was positive for vimentin. Recurrence was noted 7 mo after surgery. After chemotherapy, the recurrent tumor was resected surgically, and histopathological examination showed similar findings to those of the primary tumor. To our knowledge, this is the first report of SEF of the liver with tumor invasion of the IVC.

Approaches to reconstruction of inferior vena cava by ex vivo liver resection and autotransplantation in 114 patients with hepatic alveolar echinococcosis

BACKGROUND Hepatic alveolar echinococcosis(AE)is most commonly found in retrohepatic inferior vena cava(RHIVC).Ex vivo liver resection and autotransplantation(ELRA)can better realize the radical resection of end-stage hepatic AE with severely compromised hepatocaval confluences,and reconstruction of the affected vessels.Currently,there is a scarcity of information regarding RHIVC reconstruction in ELRA.AIM To propose reasonable RHICV reconstruction strategies for ex vivo liver resection and autotransplantation.METHODS We retrospectively summarized the clinical data of 114 patients diagnosed with hepatic AE who treated by ELRA in our department.A total of 114 patients were divided into three groups according to the different reconstruction methods of RHIVC:Group A with original RHIVC being repaired and reconstructed(n=64),group B with RHIVC being replaced(n=43),and group C with RHIVC being resected without reconstruction(n=7).The clinical data of patients,including the operation time,anhepatic phase,intraoperative blood loss,complications and postoperative hospital stay,were analyzed and the patients were routinely followed up.The normally distributed continuous variables were expressed as means±SD,whereas the abnormally distributed ones were expressed as median and analyzed by analysis of variance.Survival curve was plotted by the Kaplan-Meier method.RESULTS All patients were routinely followed up for a median duration of 52(range,12-125)mo.The 30 d mortality rate was 7.0%(8/114)and 7 patients died within 90 d.Among all subjects,the inferior vena cava(IVC)-related complication rates were 17.5%(11/63)in group A and 16.3%(7/43)in group B.IVC stenosis was found in 12 patients(10.5%),whereas thrombus was formed in 6 patients(5.3%).Twenty-two patients had grade III or higher complications,with the complication rates being 17.2%,16.3%,and 57.1%in the three groups.The average postoperative hospital stay in the three groups was 32.3±19.8,26.7±18.2,and 51.3±29.4 d(P=0.03),respectively.CONCLUSION ELRA can be considered a safe and feasible option for end-stage hepatic AE patients with RHIVC infiltration.The RHIVC reconstruction methods should be selected appropriately depending on the defect degree of AE lesions in IVC lumen.The RHIVC resection without any reconstruction method should be considered with caution.

Correlation of Inferior Vena Cava Respiratory Variability Index with Central Venous Pressure and Hemodynamic Parameters in Ventilated Pigs with Septic Shock

Septic shock is a common critical condition, for which effective early fluid resuscitation is the therapeutic focus. According to the 2008 international guidelines for management of severe sepsis and septic shock, resuscitation should achieve a central venous pressure （CVP） of 8-12 mmHg within the first 6 h. However, it is still uncertain about the sensitivity and specificity of CVP in reflecting the cardiac preload. Ultrasonography is a simple, rapid, non-invasive, and repeatable method for the measurement of sensitivity and specificity of CVP and has thus gradually attracted the increasing attention of physicians. It was reported that ultrasonography can show the inferior vena cava diameter, respiratory variability index, and blood volume in patients with sepsis or heart failure.

Anesthetic management for intraoperative acute pulmonary embolism during inferior vena cava tumor thrombus surgery:A case report

BACKGROUND Acute pulmonary embolism(APE)is a rare and potentially life-threatening condition,even with early detection and prompt management.Intraoperative APE required specific ways for detecting since classic symptoms of APE in the awake patient could not be observed or self-reported by the patient under general anesthesia.CASE SUMMARY A 44-year-old man with a history of hepatic cell carcinoma was admitted for radical nephrectomy and tumor thrombectomy due to a newly found kidney tumor with inferior vena cava(IVC)tumor thrombus.APE that occurred during tumor thrombectomy with hypercapnia and desaturation.The capnography combined with the transesophageal echocardiography(TEE)provided a crucial differential diagnosis during the operation.The patient was continuously managed with aggressive intravenous fluid resuscitation and blood transfusion under continuous cardiac output monitoring to maintain hemodynamic stability.He completed the surgery under stable hemodynamics and was extubated after percutaneous mechanical thrombectomy by a certified cardiologist.There were no significant symptoms and signs or obvious discomfort in the patient’s self-report during visits to the general ward.CONCLUSION Under general anesthesia for IVC tumor thrombus surgery,a sudden decrease in end-tidal carbon dioxide is the initial indicator of APE,which occurs before hemodynamic changes.When intraoperative APE is suspected,TEE is useful in the diagnosis and monitoring before computer tomography pulmonary angiogram.Timely clinical impression and supportive treatment and intervention should be conducted to obtain a better prognosis.

Resection and reconstruction of the inferior vena cava for neoplasms

AIM: To evaluate the results of an aggressive surgical approach of resection and reconstruction of the inferior vena cava (IVC).

Comparative outcomes of Inferior Vena Cava filters placed at bedside using digital radiography versus conventional fluoroscopy

Purpose:To retrospectively assess the outcomes of Inferior Vena Cava(IVC)filters placed in critically ill patients in the ICU at bedside using digital radiograph(DR)guidance with previous cross-sectional imaging for planning,compared to IVC filters placed by conventional fluoroscopy(CF).Method and materials:The cohort consisted of 129 IVC filter placements;48 placed at bedside and 81 placed conventionally from July 2015 to September 2016.Patient demographics,indication,radiation exposures,access site,procedural duration,dwell time,and complications were identified by the EMR.IVC Filter positioning with measurements of tip to renal vein distance and lateral filter tilt were performed when cavograms or post placement CTs were available for review.Statistical analysis was performed using Stata IC 11.2.Results:Technical success of the procedure was 100% in both groups.Procedural duration was longer at the bedside lasting 14.5+/-10.2 versus 6.7+/-6.0 min(p<0.0001).The bedside DR group had a median radiation exposure of 25 mGy(15-35)and the CF group had mean radiation exposure of 256.94 mGy+/-158.6.There was no significant difference in distance of IVC tip to renal vein(p=0.31),mispositioning(p=0.59),degree of filter tilt(p=0.33),or rate of complications(p=0.65)between the two groups.Conclusion:IVCF placement at the bedside using DR is comparable to CF with no statistical difference in outcomes based on IVCF positioning,degree of lateral tilt or removal issues.It decreased radiation dose,but with overall increased procedural time.

Contemporary techniques and outcomes of surgery for locally advanced renal cell carcinoma with focus on inferior vena cava thrombectomy:The value of a multidisciplinary team

Objective:To report the outcomes of surgery for a contemporary series of patients with locally advanced non-metastatic renal cell carcinoma(RCC)treated at a referral academic centre,focusing on technical nuances and on the value of a multidisciplinary team.Methods:We queried our prospective institutional database to identify patients undergoing surgical treatment for locally advanced(cT3-T4 N0-1 M0)renal masses suspected of RCC at our centre between January 2017 and December 2020.Results:Overall,32 patients were included in the analytic cohort.Of these,12(37.5%)tumours were staged as cT3a,8(25.0%)as cT3b,5(15.6%)as cT3c,and 7(21.9%)as cT4;6(18.8%)patients had preoperative evidence of lymph node involvement.Nine(28.1%)patients underwent nephron-sparing surgery while 23(71.9%)received radical nephrectomy.A template-based lymphadenectomy was performed in 12 cases,with evidence of disease in 3(25.0%)at definitive histopathological analysis.Four cases of RCC with level IV inferior vena cava thrombosis were successfully treated using liver transplant techniques without the need for extracorporeal circulation.While intraoperative complications were recorded in 3(9.4%)patients,no postoperative major complications(Clavien-Dindo3)were observed.At histopathological analysis,2(6.2%)patients who underwent partial nephrectomy harboured oncocytoma,while the most common malignant histotype was clear cell RCC(62.5%),with a median Leibovich score of 6(interquartile range 5e7).Conclusion:Locally advanced RCC is a complex and heterogenous disease posing several challenges to surgical teams.Our experience confirms that provided careful patient selection,surgery in experienced hands can achieve favourable perioperative,oncological,and functional outcomes.

Deep vein thrombosis in patient with left-sided inferior vena cava draining into the hemiazygos vein: A case report

BACKGROUND Abnormalities of the inferior vena cava(IVC)are uncommon,and in many cases they are asymptomatic.Even so,it is vital that clinicians be aware of such anomalies prior to surgery in affected individuals.In the present report,we describe a rare anatomical variation of the IVC.CASE SUMMARY A 66-year-old male was admitted to the hospital due to deep vein thrombosis of the right lower extremity.Upon contrast-enhanced computed tomography imaging,we found that this patient presented with a case of left-sided IVC draining into the hemiazygos vein,while his hepatic vein was directly draining into the atrium.CONCLUSION Cases of left-sided IVC can increase patient susceptibility to thromboembolism owing to the resultant changes in blood flow and/or associated vascular compression.

Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis:A case report and review of the literature

BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.

High Inferior Vena Cava Thrombosis in a 16-year-old Postpartum Patient:A Case Report

Postpartum inferior vena cava (IVC) thrombosis is a rare,but potentially life-threatening disorder.Here we reported one case of the youngest woman to date who presented with massive IVC thrombus extending from deep veins of the right leg to the level of the 11th thoracic vertebra,associated with asymptomatic pulmonary embolism.

Surgery for hepatocellular carcinoma with tumor thrombosis in inferior vena cava: A case report

BACKGROUND Hepatocellular carcinoma(HCC)accompanied by a tumor thrombus is very common.However,the treatment strategy is controversial and varies by the location of the thrombus.CASE SUMMARY We report herein a case of HCC with a tumor thrombus in the suprahepatic inferior vena cava(IVC),which was successfully treated by hepatectomy combined with thrombectomy following sorafenib chemotherapy.A 47-year-old woman with chronic hepatitis was diagnosed with HCC.Computed tomography and magnetic resonance imaging showed that the tumor lesion was located in the right half of the liver,and a tumor thrombus was detected in the suprahepatic IVC near the right atrium.After multi-departmental discussion and patient informed consent,right major hepatectomy and total removal of the tumor thrombus were successfully performed under cardiopulmonary bypass.There were no serious complications after surgery.Following sorafenib treatment,no recurrence has been detected so far(11 mo later).CONCLUSION Surgical treatment followed by adjuvant sorafenib therapy might be an acceptable choice for HCC patients with tumor thrombosis in the IVC.

Successful management of infected right iliac pseudoaneurysm caused by penetration of migrated inferior vena cava filter:A case report

BACKGROUND Indwelling inferior vena cava(IVC)filters might cause various complications,including filter penetration,filter fracture,filter migration,and thrombosis of the IVC.Penetration and migration complications are common,while a caudal migrated double-basket filter with associated infected iliac pseudoaneurysm has seldom been reported.CASE SUMMARY We report a 64-year-old female admitted for sudden onset of severe right abdominal pain after IVC filter placement for 3 mo.The patient had a history of failed endovascular IVC filter retrieval.Computed tomography showed that the retrieval hook of the filter penetrated the right common iliac artery and vein,leading to right iliac artery pseudoaneurysm accompanied by right ureteral obstruction with ipsilateral hydronephrosis,and bilateral iliac veins were occluded.Emergency open repair was performed to remove the IVC filter,the right iliac pseudoaneurysm,and the compromised segments of the iliac veins and IVC with right common iliac artery reconstruction.Staphylococcus aureus was isolated from the tissue culture.The patient was discharged on postoperative day 12 with anticoagulation therapy and antibiotic therapy after discharge.Six-month follow-up computed tomography revealed that the right common iliac artery was patent,and only mild hydronephrosis was detected.CONCLUSION An indwelling IVC filter,even‘embedded’within organized thrombus,could still cause life-threatening complications.Open procedures remain the last resort for IVC filters with severe complications.

Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

Background:The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow(AnPBF)in single-ventricle(SV)and interrupted inferior vena cava(IVC)heart disease is still uncertain as yet.Methods:We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018,perioperative,operative and postoperative characteristics were recorded.Results:A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years(range 0.5–24.7 years),of which 12(66.7%)were male and 6(33.3%)were female.The mean saturation was 76.2±8.5%in preoperative period and 94.2±2.2%in postoperative period.All patients had kept AnPBF.The median duration of mechanical ventilation was 12 h(range 2.5–22.5 h)and the median duration of pleural drainage was 5 days(range 2–27 days).The median hospital stay was 9 days(range 6–70 days).There was no operative death and no mortality was seen in early postoperative period.Follow-up was 100%completed,with an average follow-up period of 6.1±2.7 years(range 1–11 years).4 patients died during the follow-up.The overall 5 and 10 years’survival rates estimated by Kaplan-Meier method were 88%and 68%,respectively.Although there were no significant differences in the duration of postoperative follow-up between the death group and the survival group(p>0.05),the major systemic ventricular end-diastolic diameter(SVEDD)(p=0.018)and the degree of AVVR(p=0.001)showed significant difference between the two groups.The diameters of main pulmonary artery showed significant growth in both the death group(p=0.015)and the survival group(p=0.012)over time.SVEDD had no significant increase in the survival group(p=0.665)but was significantly larger in the death group(p=0.014).Multivariable risk factors of late mortality in patients treated with Kawashima procedure were follow-up AVVR(p=0.044;HR:3.124;95%CI:1.030–9.473)and SVEDD(p=0.031;HR:9.766;95%CI:1.226–77.8).14 patients(100%)were all in New York Heart Association(NYHA)functional class I and the mean saturation was 93±2%at last follow-up.Only one patient finished Fontan completion.Conclusions:The Kawashima procedure with AnPBF can be safely performed with acceptable early and long outcomes.Although some previous studies have shown the risk of pulmonary arteriovenous malformations(PAVMS)after Kawashima procedure in the mid-and long-term,our findings are in contradiction with it.No PAVMs occurred in all the survivors.Kawashima procedure with open AnPBF may be a good option for unsuitable Fontan candidates.