Fistula formation is a very rare complication after transjugular intrahepatic portosystemic shunt (TIPS) placement.1 PubMed search shows only 2 cases of such arterioportal fistula, one between the right hepatic artery and portal vein2 and another between the left hepatic artery and portal vein.3 However, there are no reported cases documenting fistula development between the celiac artery and portal vein after TIPS placement. Despite its rarity, it is important to consider this unusual complication when a patient presents with fluid overload symptoms after TIPS.
A 61-year-old man with a medical history significant for cirrhosis secondary to hepatitis C infection and alcoholism, esophageal varices, recurrent ascites, and hypertension presented to the hospital with abdominal distension and lower extremity edema for 3 weeks. The patient underwent TIPS placement 5 months ago for acute variceal bleeding with successful embolization of varices. The portosystemic gradient decreased from 33 to 11 mm Hg after TIPS placement, and angiography showed brisk flow through the stent with no other shunting. He also reported the resolution of ascites postprocedure.
On admission, his vitals were normal. Examination revealed a distended abdomen with pitting abdominal wall and bilateral lower extremity edema extending up to the thighs. Initial laboratory results showed an elevated brain natriuretic peptide of 375 pg/mL, international normalized ratio (INR) of 1.37, and prothrombin time of 14.6 seconds. The rest of the laboratory results were normal, including liver enzymes and urine protein. Chest x-ray was suggestive of mild pulmonary vascular congestion, and an ultrasound of the abdomen failed to reveal any ascitic fluid. The patient was started on a diuretic regimen comprising furosemide and spironolactone with some improvement in symptoms. An echocardiogram was obtained the next day which showed a normal left ventricular ejection fraction of 55%–60%, a dilated right ventricle (RV), and an elevated RV systolic pressure of 53 mm Hg. A previous echocardiogram obtained 5 months ago (3 days after the TIPS procedure) showed similar left ventricular ejection fraction of 60% but normal RV size and elevated RV systolic pressure of 47 mm Hg. The patient did not have any history of heart failure, pulmonary hypertension, chronic lung disease, or chronic inflammatory condition. A ventilation/perfusion scan was obtained which was suggestive of a low probability of chronic venous thromboembolism, ruling out its role in elevated RV/pulmonary artery pressures.
The patient then underwent right heart catheterization which revealed an increase in cardiac output 11.05 L/min, cardiac index 5.52 L/min/m2, as well as elevated RV pressure (systolic/diastolic/mean 75/15/35 mm Hg), pulmonary artery pressure (systolic/diastolic/mean 74/35/53 mm Hg), and pulmonary capillary wedge pressure (systolic/diastolic/mean 48/50/39 mm Hg). The right atrial oxygen saturation was noted to be high at approximately 85%. Oxygen saturation in the inferior vena cava (IVC) was also found to be high at 85%, which is consistent with the right atrial saturation. The O2 saturation cephalad to the TIPS catheter was slightly higher than O2 saturation caudal to the TIPS catheter which prompted concern over an arteriovenous fistula (AVF). For further evaluation, a hepatic angiogram was obtained that revealed a collateral vessel arising from the origin of the celiac artery communicating with the portal vein. When the catheter was further advanced into this collateral vessel, numerous additional vessels arising from this collateral vessel and communicating with the portal vein were found that resulted in a functional AVF (Figure 1). The AVF was successfully embolized with coils which led to markedly reduced contrast shunting through the liver (Figure 2). The patient was discharged after 5 additional days of treatment with diuretics, and improvement in edema was noted. On outpatient follow-up at 2 months after discharge, the patient reported no recurrence of abdominal wall edema, with mild lower extremity edema well controlled on a diuretic regimen.
Cirrhosis results in a hyperdynamic circulatory state with elevated cardiac output and decreased peripheral vascular resistance.4 TIPS is an important therapeutic tool available to decrease portal hypertension in patients with cirrhosis by creating an artificial fistula between the hepatic vein and portal vein. There are a wide variety of complications that can be encountered with TIPS including but not limited to new or worsening of hepatic encephalopathy, stent occlusion, thrombosis, stent migration, transcapsular puncture, hemolysis, hemorrhage, infection, and rarely fistula formtion.1
Right-sided heart failure is a well-known complication of TIPS, and the incidence of symptomatic right heart failure after TIPS placement was estimated to be 0.9% in a recently published study.5 However, the study is limited to symptom onset within 7 days of TIPS placement. TIPS has been shown to cause an increase in cardiac output and cardiac index and a decrease in systemic vascular resistance and systemic arterial blood pressure.6 The changes in hemodynamic parameters will be more pronounced if there is a concomitant arteriovenous fistula.
As mentioned above, fistula is a rare complication of TIPS, and the mechanism of injury in our case was thought to be due to injury to the celiac trunk during the placement of the TIPS shunt.1 Review of the literature did not show any similar cases involving the celiac trunk; however, 2 cases of hepatic artery and portal venous fistulas were reported based on PubMed search.2,3 Sedat et al reported a case of a fistula between the right hepatic artery and portal vein presenting as liver failure, whereas Brophy et al reported another similar case of a fistula between the left hepatic artery and portal vein presenting with variceal bleeding and intractable ascites.2,3 This case highlights the importance of keeping unusual complications in the differentials when a patient develops congestive heart failure after TIPS placement, and appropriate management of them can result in significant improvement in symptoms.
Author contributions: PR Kathi wrote the manuscript. N. Thammineni edited the manuscript. K. Dhillon, S. Kundumadam, and S. Goyal reviewed the manuscript. S. Goyal is the article guarantor.
Financial disclosure: None to report.
Informed consent was obtained for this case report.
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2. Sedat J, Padovani B, Chanalet S. Intrahepatic fistula after portosystemic transjugular shunt placement complications from enteroclysis tube insertion needle core biopsy of mammographic lesions. Am J Roentgenol. 1995;164(1):259.
3. Brophy DP, Vrachliotis T, Chavali R, Rabkin DJ. SCVIR annual meeting film panel session: Diagnosis and discussion of case 2: Left hepatic arterioportal fistula. J Vasc Interv Radiol. 2001;12(4):535–9.
4. Braverman AC, Steiner MA, Picus D, White H. High-output congestive heart failure following transjugular intrahepatic portal-systemic shunting. Chest. 1995;107(5):1467–9.
5. Modha K, Kapoor B, Lopez R, Sands MJ, Carey W. Symptomatic heart failure after transjugular intrahepatic portosystemic shunt placement: Incidence, outcomes, and predictors. Cardiovasc Intervent Radiol. 2018;41(4):564–71.
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6. Saugel B, Mair S, Meidert AS, et al. The effects of transjugular intrahepatic portosystemic stent shunt on systemic cardiocirculatory parameters. J Crit Care. 2014;29(6):1001–5.