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Journal of Medical Case Reports Jul 2022Recombinant adenoviral vector vaccines against severe acute respiratory syndrome coronavirus 2 have been observed to be associated with vaccine-induced immune thrombotic...
Transjugular intrahepatic portosystemic shunt, local thrombaspiration, and lysis for management of fulminant portomesenteric thrombosis and atraumatic splenic rupture due to vector-vaccine-induced thrombotic thrombocytopenia: a case report.
INTRODUCTION
Recombinant adenoviral vector vaccines against severe acute respiratory syndrome coronavirus 2 have been observed to be associated with vaccine-induced immune thrombotic thrombocytopenia. Though vaccine-induced immune thrombotic thrombocytopenia is a rare complication after vaccination with recombinant adenoviral vector vaccines, it can lead to severe complications. In vaccine-induced immune thrombotic thrombocytopenia, the vector vaccine induces heparin-independent production of platelet factor 4 autoantibodies, resulting in platelet activation and aggregation. Therefore, patients suffering from vaccine-induced immune thrombotic thrombocytopenia particularly present with signs of arterial or venous thrombosis, often at atypical sites, but also signs of bleeding due to disseminated intravascular coagulation and severe thrombocytopenia. We describe herein a rare case of fulminant portomesenteric thrombosis and atraumatic splenic rupture due to vaccine-induced immune thrombotic thrombocytopenia. This case report presents the diagnosis and treatment of a healthy 29-year-old male Caucasian patient suffering from an extended portomesenteric thrombosis associated with atraumatic splenic rupture due to vaccine-induced immune thrombotic thrombocytopenia after the first dose of an adenoviral vector vaccine against severe acute respiratory syndrome coronavirus 2 [ChAdOx1 nCoV-19 (AZD1222)]. Therapeutic management of vaccine-induced immune thrombotic thrombocytopenia initially focused on systemic anticoagulation avoiding heparin and the application of steroids and intravenous immune globulins as per the recommendations of international societies of hematology and hemostaseology. Owing to the atraumatic splenic rupture and extended portomesenteric thrombosis, successful management of this case required splenectomy with additional placement of a transjugular intrahepatic portosystemic shunt to perform local thrombaspiration, plus repeated local lysis to reconstitute hepatopetal blood flow.
CONCLUSION
The complexity and wide spectrum of the clinical picture in patients suffering from vaccine-induced immune thrombotic thrombocytopenia demand an early interdisciplinary diagnostic and therapeutic approach. Severe cases of portomesenteric thrombosis in vaccine-induced immune thrombotic thrombocytopenia, refractory to conservative management, may require additional placement of a transjugular intrahepatic portosystemic shunt, thrombaspiration, thrombolysis, and surgical intervention for effective management.
Topics: Adult; COVID-19; ChAdOx1 nCoV-19; Heparin; Humans; Male; Portasystemic Shunt, Transjugular Intrahepatic; Purpura, Thrombocytopenic, Idiopathic; Splenic Rupture; Thrombocytopenia; Thrombosis; Vaccines
PubMed: 35821156
DOI: 10.1186/s13256-022-03464-x -
Liver Transplantation : Official... Nov 20061. Diagnosis of Budd-Chiari syndrome can be made on the basis of radiological imaging alone without the need for liver biopsy. 2. Ultrasonography, computed tomography,... (Review)
Review
1. Diagnosis of Budd-Chiari syndrome can be made on the basis of radiological imaging alone without the need for liver biopsy. 2. Ultrasonography, computed tomography, and magnetic resonance imaging all show various degrees of occlusion of the hepatic veins and/or inferior vena cava. Hypertrophy of the caudate lobe may also be seen. 3. Computed tomographic and magnetic resonance imaging give a better idea of hepatic perfusion. Image reconstruction of the inferior vena cava is also possible. 4. Hepatic venography demonstrates a spiderweb pattern diagnostic of Budd-Chiari syndrome. 5. Pressure measurements in the portal vein and infrahepatic inferior vena cava are necessary to determine whether a surgical portosystemic shunt will be successful. 6. Transjugular intrahepatic portosystemic shunt provides definitive treatment in many patients; this is not discussed.
Topics: Anticoagulants; Budd-Chiari Syndrome; Humans; Liver Transplantation; Portasystemic Shunt, Transjugular Intrahepatic; Radiography; Stents
PubMed: 17051559
DOI: 10.1002/lt.20939 -
JACC. Cardiovascular Interventions Jan 2010More than 1 in 1,000 patients in the U.S. has end-stage renal disease, and most patients who require renal-replacement therapy undergo hemodialysis. By the year 2020,... (Review)
Review
More than 1 in 1,000 patients in the U.S. has end-stage renal disease, and most patients who require renal-replacement therapy undergo hemodialysis. By the year 2020, more than 750,000 patients are expected to have end-stage renal disease, and over 500,000 will require hemodialysis. The greatest limitation of hemodialysis is the finite durability of hemodialysis accesses, which on average remain patent for <3 years but are the lifeline for hemodialysis patients. Catheter-based interventions are successful in restoring flow in more than 80% of hemodialysis accesses that undergo thrombosis and have replaced surgical revision as the treatment of choice for failing or thrombosed accesses. Catheter-based interventions have improved the quality of life for hemodialysis patients by reducing the need for temporary hemodialysis catheters and have prolonged total survival time by preserving existing access sites and by saving venous segments for future access creation. This review discusses the pathophysiology of dialysis access failure, presents the success rates of catheter-based treatments, and illustrates the interventional approaches for treating failing and thrombosed fistulas and grafts.
Topics: Angioplasty, Balloon; Arteriovenous Shunt, Surgical; Blood Vessel Prosthesis Implantation; Cardiovascular Agents; Graft Occlusion, Vascular; Humans; Kidney Failure, Chronic; Phlebography; Quality of Life; Radiography, Interventional; Renal Dialysis; Thrombectomy; Thrombosis; Time Factors; Treatment Outcome; Upper Extremity; Vascular Patency
PubMed: 20129561
DOI: 10.1016/j.jcin.2009.10.021 -
Clinical Journal of the American... Aug 2009Patients with end stage renal disease (ESRD) are often prescribed antiplatelet medications. However, these patients are also at increased risk of bleeding compared with... (Review)
Review
BACKGROUND AND OBJECTIVES
Patients with end stage renal disease (ESRD) are often prescribed antiplatelet medications. However, these patients are also at increased risk of bleeding compared with the general population, and an aim was made to quantify this risk with antiplatelet agents.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS
A systematic review of the literature (Medline, EMBASE, Cochrane CENTRAL and Google Scholar databases) was done to determine the bleeding risk in ESRD patients prescribed antiplatelet therapy. The secondary outcome was the effect on access thrombosis. All case series, cohort studies and clinical trials were considered if they included ten or more ESRD patients, assessed bleeding risk with antiplatelet agents, and lasted for more than 3 mo.
RESULTS
Sixteen studies, including 40,676 patients, were identified that met predefined inclusion criteria. Due to study heterogeneity and weaknesses in methodology, bleeding rates were not pooled across studies. However, the bleeding risk appears to be increased for hemodialysis patients treated with combination antiplatelet therapy. The results are mixed for studies using a single antiplatelet agent. Antiplatelet agents appear to be effective in preventing shunt and central venous catheter thrombosis, but not for preventing thrombosis of arteriovenous grafts.
CONCLUSION
The risks and benefits of antiplatelet agents in ESRD patients remain poorly defined. Until a clinical trial addresses this in the dialysis population, individual risk stratification taking into account the increased risk of bleeding should be considered before initiating antiplatelet agents, especially in combination therapy.
Topics: Arteriovenous Shunt, Surgical; Blood Vessel Prosthesis Implantation; Catheterization, Central Venous; Drug Therapy, Combination; Hemorrhage; Humans; Kidney Failure, Chronic; Platelet Aggregation Inhibitors; Renal Dialysis; Risk Assessment; Thrombosis; Treatment Outcome
PubMed: 19578002
DOI: 10.2215/CJN.00810209 -
Medical Principles and Practice :... 2022Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical...
OBJECTIVE
Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical manifestations and the risk factors for development of EHPVT in pediatric patients.
SUBJECTS AND METHODS
This was a single-center retrospective cohort study. A total of 12 children (6 boys and 6 girls) took part in the study. We noted the clinical presentations and the predisposing risk factors for development of EHPVT in all patients. In addition, as all of them had undergone an esophagogastroduodenoscopy for detection and grading of esophageal varices as part of the treatment algorithm, we analyzed the endoscopic findings and the therapeutic approach.
RESULTS
The median age of subjects at diagnosis was 3.5 years (range: 1-17 years). The most frequent initial clinical manifestation was upper gastrointestinal bleeding (6 cases, 50.0%) followed by splenomegaly (3 cases, 25.0%). The most frequent systemic risk factor for EHPVT was presence of inherited prothrombotic disorder (10 cases, 83.3%), and the most common local risk factor for EHPVT was umbilical vein catheterization (5 cases, 41.7%). Esophageal varices were revealed in all the study participants, and in the most cases, they were grade ≥2. Propranolol was used as primary or secondary prophylaxis in 7 children (58.3%), and in 5 children (41.7%), a shunt was performed (Meso-Rex bypass in 3 children and splenorenal shunt in 2 children).
CONCLUSION
Patients with known systemic or local risk factors for EHPVT are indicated for proactive ultrasound screening for early diagnosis and timely management.
Topics: Male; Female; Child; Humans; Infant; Child, Preschool; Adolescent; Portal Vein; Esophageal and Gastric Varices; Retrospective Studies; Venous Thrombosis; Risk Factors; Gastrointestinal Hemorrhage
PubMed: 36215953
DOI: 10.1159/000527247 -
Annals of Hepatology 2016Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization...
Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization or transplantation. Here, a 22 year old female with angiographically proven BCS secondary to JAK2/V617F positive Polycythemia vera on therapeutic warfarin presented with acute liver failure (ALF). Imaging revealed a new, near complete thrombotic occlusion of the main portal vein with extension into the superior mesenteric vein. An emergent direct intrahepatic portocaval shunt (DIPS) was created and liver function promptly normalized. She has been maintained on rivaroxaban since that time. Serial assessment over 1 year demonstrated continued shunt patency and improved flow in the mesenteric vasculature on ultrasound as well as normal liver function. DIPS is a viable alternative in the treatment of ALF from BCS when standard recanalization is not feasible. Improved blood flow may also improve portal/mesenteric clot burden. While further investigation is needed, new targeted anticoagulants may be viable as a long term anticoagulation strategy.
Topics: Anticoagulants; Biopsy; Budd-Chiari Syndrome; Drug Substitution; Female; Humans; International Normalized Ratio; Janus Kinase 2; Liver Failure, Acute; Mutation; Phlebography; Polycythemia Vera; Portacaval Shunt, Surgical; Portal Vein; Rivaroxaban; Treatment Outcome; Vascular Patency; Venous Thrombosis; Warfarin; Young Adult
PubMed: 26626649
DOI: 10.5604/16652681.1184288 -
Liver Transplantation : Official... Mar 2003Transjugular intrahepatic portosystemic shunts (TIPS) have been used in the treatment of complications of portal hypertension. TIPS is used for the control of acute... (Review)
Review
Transjugular intrahepatic portosystemic shunts (TIPS) have been used in the treatment of complications of portal hypertension. TIPS is used for the control of acute variceal bleeding and for the prevention of vericeal rebleeding when pharmacologic therapy and endoscopic therapy have failed. Patients with refractory ascites with adequate hepatic reserve and renal function who fail to respond to large volume paracentesis may be reasonable candidates for TIPS. Promising indications for TIPS are Budd-Chiari syndrome uncontrolled by medical therapy, severe portal hypertensive gastropathy, refractory hepatic hydrothorax, and hepatorenal syndrome. TIPS cannot be recommended for preoperative portal decompression solely to facilitate liver transplantation. Special care should be taken to insure proper placement of the stent to avoid increasing the technical difficulty of the transplantation procedure. The major limiting factors for TIPS success are shunt dysfunction and hepatic encephalopathy. Because shunt stenosis is the most important cause of recurrent complications of portal hypertension, a surveillance program to monitor shunt patency is mandatory. The MELD score may be useful in predicting post-TIPS survival, and also in counseling patients and their families.
Topics: Budd-Chiari Syndrome; Humans; Hypertension, Portal; Portasystemic Shunt, Transjugular Intrahepatic; Randomized Controlled Trials as Topic
PubMed: 12619016
DOI: 10.1053/jlts.2003.50045 -
World Journal of Gastroenterology Apr 2020Spontaneous porto-systemic shunts (SPSS) are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates, probably as a consequence of... (Review)
Review
Spontaneous porto-systemic shunts (SPSS) are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates, probably as a consequence of worsening portal hypertension, but without achieving an effective protection against cirrhosis' complications. Several types of SPSS have been described in the literature, each one associated with different clinical manifestations. In particular, recurrent or persistent hepatic encephalopathy is more frequent in patients with splenorenal shunt, while the presence of gastric varices and consequently the incidence of variceal bleeding is more common in gastrorenal shunt. In the advanced stage, the presence of large SPSS can lead to the so called "portosystemic shunt syndrome", characterized by a progressive deterioration of hepatic function, hepatic encephalopathy and, sometimes, portal vein thrombosis. The detection of SPSS in patients with liver cirrhosis is recommended in order to prevent or treat recurrent hepatic encephalopathy or variceal bleeding.
Topics: Collateral Circulation; Esophageal and Gastric Varices; Gastrointestinal Hemorrhage; Hepatic Encephalopathy; Humans; Incidence; Liver; Liver Cirrhosis; Liver Function Tests; Portal System; Syndrome; Treatment Outcome; Venous Thrombosis
PubMed: 32351289
DOI: 10.3748/wjg.v26.i15.1726 -
World Journal of Gastroenterology Aug 2019Portal vein thrombosis (PVT) represents a well-known complication during the natural course of liver cirrhosis (LC), ranging from asymptomatic cases to life-threating... (Review)
Review
Portal vein thrombosis (PVT) represents a well-known complication during the natural course of liver cirrhosis (LC), ranging from asymptomatic cases to life-threating conditions related to portal hypertension and hepatic decompensation. Portal flow stasis, complex acquired hypercoagulable disorders and exogenous factors leading to endothelial dysfunction have emerged as key factors for PVT development. However, PVT occurrence remains unpredictable and many issues regarding its natural history, prognostic significance and treatment are still elusive. In particular although spontaneous resolution or disease stability occur in most cases of PVT, factors predisposing to disease progression or recurrence after spontaneous recanalization are not clarified as yet. Moreover, PVT impact on LC outcome is still debated, as PVT may represent itself a consequence of liver fibrosis and hepatic dysfunction progression. Anticoagulation and transjugular intrahepatic portosystemic shunt are considered safe and effective in this setting and are recommended in selected cases, even if the safer therapeutic option and the optimal therapy duration are still unknown. Nevertheless, their impact on mortality rates should be addressed more extensively. In this review we present the most debated questions regarding PVT, whose answers should come from prospective cohort studies and large sample-size randomized trials.
Topics: Administration, Oral; Anticoagulants; Disease Progression; Female; Humans; Hypertension, Portal; Liver Cirrhosis; Patient Selection; Portal Vein; Portasystemic Shunt, Transjugular Intrahepatic; Prognosis; Prospective Studies; Time Factors; Treatment Outcome; Venous Thrombosis
PubMed: 31496623
DOI: 10.3748/wjg.v25.i31.4437 -
European Respiratory Review : An... Dec 2012Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.
Topics: Antihypertensive Agents; Bosentan; Clinical Trials as Topic; Comorbidity; Down Syndrome; Eisenmenger Complex; Endothelium, Vascular; Epoprostenol; Health Behavior; Heart Defects, Congenital; Heart Transplantation; Humans; Hypertension, Pulmonary; Lung Transplantation; Oxygen Inhalation Therapy; Phosphodiesterase 5 Inhibitors; Piperazines; Practice Guidelines as Topic; Pulmonary Circulation; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Thrombosis
PubMed: 23204121
DOI: 10.1183/09059180.00004712