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Acute Budd-Chiari Syndrome with Complete Portal Vein Thrombosis complicated by Hepato-Renal Syndrome Treated Successfully by Emergent TIPS With Rheolytic Thrombectomy

Published:November 18, 2022DOI:https://doi.org/10.1016/j.jceh.2022.11.007

      Abstract

      We described a case of a 73 years-old female, admitted to the emergency department with acute hepatic and renal failure (hepato-renal syndrome, HRS) due to acute Budd-Chiari syndrome associated with complete portal vein thrombosis (BCS-PVT) for unknown cause. Despite the initial therapy with anticoagulants, a sudden impairment of the renal function requiring hemodialysis was observed. Hepatic transplant was excluded for patient age and clinical conditions. Thus, the patient was successfully treated by emergent transjugular intrahepatic portosystemic shunt (TIPS) previous rheolytic thrombectomy of the PVT with AngioJet Ultra PE Thrombectomy System (Boston Scientific, Marlborough, MA, USA). After the procedure sudden resolution of the HRS was observed and the patient is alive 13 months after hospital dismission with no TIPS dysfunction.
      In conclusion, emergent extended TIPS with the usage of rheolytic thrombectomy device in patient with acute BCS-PVT complicated by HRS is feasible by experienced operators and provide resolution of the HRS.

      Keywords

      Abbreviations

      BCS:
      Budd-Chiari syndrome.
      CECT:
      contrast-enhanced computed tomography.
      HRS:
      hepato-renal syndrome.
      HV:
      hepatic veins.
      INR:
      international normalized ratio.
      IVC:
      inferior vena cava.
      PVT:
      portal vein thrombosis
      TIPS:
      transjugular intrahepatic portosystemic shunt.
      PSS:
      surgical portosystemic shunting.
      PTFE:
      polytetrafluoroethylene.
      To the Editor

      Declaration of Interest statement

      none.

      Funding source

      none.

      Author contributions

      Emanuele Michieletti: Conceptualization, Methodology, Supervision, Validation, Writing – original draft. Flavio Cesare Bodini: Conceptualization, Methodology, Supervision, Validation. Nicola Morelli: Data curation, Formal analysis, Investigation. Beatrice Rossi: Data curation, Formal analysis, Investigation. Margherita Bossalini: Data curation, Formal analysis, Investigation. Davide Colombi: Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Supervision, Validation, Visualization, Writing – original draft.
      me (BCS) is a rare potentially life-threatening disease caused by hepatic venous outflow obstruction at any level between small hepatic veins (HV) and right atrium (RA)
      • Janssen H.L.A.
      • Garcia-Pagan J.-C.
      • Elias E.
      • Mentha G.
      • Hadengue A.
      • Valla D.-C.
      Budd-Chiari syndrome: a review by an expert panel.
      . BCS combined with portal vein thrombosis (PVT) has poorer prognosis as compared to BCS alone
      • Murad S.D.
      • Valla D.C.
      • De Groen P.C.
      • et al.
      Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis.
      . Treatment of BCS-PVT is based on a step-wise approach, with anticoagulation as first-line therapy followed by more invasive procedures (transjugular intrahepatic portosystemic shunt, TIPS; surgical portosystemic shunting, PSS)
      • Murad S.D.
      • Valla D.C.
      • De Groen P.C.
      • et al.
      Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis.
      . TIPS associated with portal vein thrombolysis or balloon pull-through angioplasty appeared to have the best outcome
      • Murad S.D.
      • Valla D.C.
      • De Groen P.C.
      • et al.
      Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis.
      ,
      • Bodini F.C.
      • Rossi S.
      • Veronese L.
      • Colombi D.
      • Michieletti E.
      Extrahepatic Portosystemic Shunt via the Coronary Vein in Noncirrhotic Chronic Portal Vein Thrombosis.
      . Here, we report a case of a patient affected by BCS-PVT who developed sudden hepato-renal syndrome (HRS) treated by emergent TIPS performed after portal vein thrombectomy through aspiration device.
      A 73 years-old female was admitted to the emergency department of our hospital with abdominal distension and pain in the upper quadrants since 15 days. The patient underwent previous hysteroannessiectomy for ectopic pregnancy and suffered hypertension, hypercholesterolemia, and hypothyroidism. Laboratory findings showed the elevated levels of bilirubin (2.3 mg/dl; normal values, NV: 0-1.1 mg/dl), of serum creatinine (3 mg/dl; NV: 0.6-1 mg/dl), and blood urea (80 mg/dl; NV: 10-50 mg/dl). Point of care ultrasound (US) detected diffuse ascites. The Child and Pugh score was class C. The abdominal contrast-enhanced computed tomography (CECT) performed one day after admission revealed caudate lobe enlargement determining combined type BCS for the obstruction of the inferior vena cava (IVC) and of the three main HVs (Fig. 1a, b); in addition, complete occlusion of the main, right, and left portal vein by fresh thrombus was detected, while both splenic and superior mesenteric vein were patent (Fig. 1c). The patient was then diagnosed with acute BCS-PVT complicated by HRS
      • Giri S.
      • Kale A.
      • Shukla A.
      Efficacy and Safety of Transjugular Intrahepatic Portosystemic Shunt in Budd-Chiari Syndrome: A Systematic Review and Meta-Analysis.
      . The patient was initially treated by intravenous unfractioned heparin, nevertheless four days after admission, was detected an impairment of the renal failure (serum creatinine, 7.1 mg/dl; blood urea, 159 mg/dl). Both PSS and hepatic transplant were unsuitable for patients age and clinical conditions. Therefore, the patient underwent extended TIPS five days after admission. The patient provided a written informed consent to the procedure. A portal phlebography confirmed complete PVT of the main, right, and left portal vein (Fig. 1d) while the caval phlebography defined the stenosis of the intrahepatic IVC and of the confluence of the three HVs. A 6F catheter (AngioJet PE catheter; Boston Scientific, Marlborough, MA, USA) was inserted in the portal system by small patency at the confluence of the right HV and the IVC. The AngioJet Ultra PE Thrombectomy System (Boston Scientific) was connected to a solution prepared with saline 500cc addicted by 5000UI of sodium heparin and 100000UI of urokinase. Then the main portal vein thrombus was pharmaco-mechanically fragmented and aspirated with manual antegrade advancements throughout the thrombotic obstruction under fluoroscopic guidance, until satisfactory reperfusion was achieved. Afterwards, two covered polytetrafluoroethylene (PTFE) Viatorr stents (Gore, Flagstaff, AZ, USA) measuring 8 mm in diameter and 70 mm in length were released coaxially, in a telescoped manner, with the distal opening of the shunt deployed at the confluence of the main portal vein with the superior mesenteric vein and the proximal opening positioned in the inferior vena cava around 2-cm below right atrium. The final portography documented the patency of the bypass (Fig. 1e). No periprocedural complications were recorded. Unfractioned heparin was suspended and progressively substituted by oral warfarin with INR target 2.5. Suddenly after both hepatic and renal function progressively improved until patient discharge 22 days after the procedure. At dismission laboratory findings were markedly improved: serum bilirubin, 1.7 mg/dl; serum creatinine, 1.2 mg/dl; blood urea, 33 mg/dl. The patient was symptom-free at follow-up visit 12 months after hospital admission. Follow-up CECT performed 13 months after the procedure showed TIPS patency (Fig. 1f), without ascites or signs of portal hypertension.
      Figure 1
      Figure 1Contrast-enhanced computed tomography axial image (a) and coronal multiplanar reconstruction image with maximum intensity projection algorithm (b) reveal the obstruction of the three hepatic veins (arrows in a) and the stenosis of the intrahepatic inferior vena cava (arrow in b) configuring combined Budd-Chiari syndrome; contrast-enhanced computed tomography with maximum intensity projection algorithm in coronal multiplanar reconstruction image (c) demonstrates complete thrombosis of the main, right, and left portal vein (arrow). Portal system phlebography image (d) shows the complete thrombosis of the main, right, and left portal vein; final portography image (e) performed after rheolytic thrombectomy of the portal vein with AngioJet Ultra PE Thrombectomy System (Boston Scientific, Marlborough, MA, USA) documents the patency of the transjugular intrahepatic portosystemic shunt (TIPS) performed through two covered Viatorr stents (Gore, Flagstaff, AZ, USA). The multiplanar reconstruction image in coronal oblique plane (f) of the contrast-enhanced computed tomography performed 13 months after the procedure, shows patency of the TIPS (arrow) without ascites or signs of portal hypertension.
      Treatment of BCS-PVT relies first on anticoagulation which potentially recanalizes the occluded vein and prevents extension of thrombosis, reduceing mortality
      • Condat B.
      • Pessione F.
      • Hillaire S.
      • et al.
      Current outcome of portal vein thrombosis in adults: Risk and benefit of anticoagulant therapy.
      . Nevertheless, previous reports described dramatic improvement of patients conditions affected by BCS-PVT complicated by severe ascites or sudden HRS, who underwent emergent TIPS
      • Verma A.
      • Sharma G.
      • Mohan S.
      • Saraswat V.A.
      • Baijal S.S.
      TIPS can be lifesaving in acute liver failure associated with portal vein and inferior vena cava thrombosis in a case of Budd Chiari syndrome due to protein S deficiency.
      ,
      • Mancuso A.
      Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins.
      . In case of BCS-PVT some technical modifications of TIPS procedure are required to overcome difficulties related to the presence of PVT. Infusion of thrombolytic agents and balloon pull-through thrombectomy have been proposed for restore patency of the main portal vein
      • Murad S.D.
      • Valla D.C.
      • De Groen P.C.
      • et al.
      Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis.
      . In the present case portal vein patency was successfully restored by rheolytic thrombectomy. This technique uses high velocity jet expulsions at the catheter tip of saline solution associated with sodium heparin and urokinase in order to enter, fragment, and aspirate thrombi. The usage of this system carries several advantages: 1. against the use of systemic thrombolytic agents (e.g tissue plasminogen activator) in patients with a potentially hemorrhagic condition, or patients who have had an invasive procedure (e.g. paracentesis), during the previous 24 hours; 2. versus balloon pull-through thrombectomy which is difficult in complete thrombosis and in some cases requires two step procedures
      • Mancuso A.
      Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins.
      • Hernández-Gea V.
      • De Gottardi A.
      • Leebeek F.W.G.
      • Rautou P.E.
      • Salem R.
      • Garcia-Pagan J.C.
      Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis.
      • Watanabe H.
      • Shinzawa H.
      • Saito T.
      • et al.
      Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction.
      . .
      In conclusion, emergent extended TIPS with the usage of rheolytic thrombectomy in patient with acute BCS-PVT complicated by HRS is feasible by experienced operators, providing resolution of the HRS.

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