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Volume: 19 Issue: 10 October 2021


Diffuse Hepatic Arterioportal Fistulas After Liver Transplantation

Dear Editor:

Arterioportal fistulas (APF) are abnormal com­munications between the hepatic artery and the portal vein.1,2 The clinical spectrum of presentation ranges from symptom-free individuals to patients with severe portal hypertension. Arterioportal fistulas could be congenital (for example, hereditary hemorrhagic telangiectasia and Ehlers-Danlos syndrome), could be idiopathic, or could be secondary to cirrhosis, hepatic neoplasm, hepatic trauma, hepatic parenchymal congestion, inflammatory or infective disease, or obstruction of hepatic vein or portal vein. In addition, APF could be iatrogenic following percutaneous liver biopsy or cholangiography. Therefore, APF can be divided into 3 different types: small peripheral intrahepatic, large central, and diffuse congenital intrahepatic.2 Here, we report a patient who developed multiple diffuse hepatic APF after liver transplant (LT) and ascites and who required transarterial embolization.

Case Report

A 53-year-old male patient was listed for LT for end-stage alcohol-related liver disease and refractory ascites. In June 2017, he received a liver graft from a 66-year-old brain dead donor who died from intracerebral hemorrhage. A liver biopsy was made at the end of the procedure in the left lobe. Early postoperative complications included kidney failure and abdominal sepsis due to Klebsiella pneumoniae. Evolution was favorable under prolonged antibiotic therapy, and the patient was discharged home 4 weeks after LT. Thereafter, on day 45, an arterial anastomotic stricture was treated by angioplasty. Angiography and computed tomography (CT) scan showed no other abnormalities at that time (Figure 1, A and B). Concomitantly, ascites persisted, requiring repeat paracenteses. Ascites fluid was a transudate, without observations of infection criteria. Histological analysis of a liver biopsy disclosed significant portal fibrosis with septa, ductal proliferation, and cholestasis.

In September 2018, because of persistent ascites, a transjugular portosystemic shunt was discussed. Cavography revealed no evidence of venous outflow obstruction, and the portosystemic gradient was 9 mm of mercury. Ultrasonography confirmed the presence of ascites and showed inversion of portal flow, and CT scan revealed multiple APF with early opacification of the portal trunk, in particular of its right branches (Figure 1, C and D). The fistulas appeared diffuse, affecting the right anterior and posterior branches, whereas the left areas did not seem to be involved. There was no portal thrombosis.

Treatment options were discussed, including retransplant or transarterial embolization. It was considered appropriate to embolize the shunts as a first option. Subsequent angiography confirmed multiple intrahepatic APF within the right lobe, and a first session of embolization with coils was performed in December 2018 (Figure 1, E and F). The patient’s postoperative course was uneventful.

In March 2021, a CT scan confirmed the absence of ascites without further significant modifications of APF; the patient did not require paracenteses.


During and after LT, the liver graft undergoes a significant number of potential procedures that can cause APF, the most frequent being liver biopsy. Nevertheless, the appearance of APF in the posttransplant setting is not common. The precise incidence is unknown, but a large retrospective series that included 1992 patients disclosed 4 cases (0.2%) of hemodynamically significant APF, from which 2 were symptomatic; all cases were localized APF.3 In the patient reported here, transplant of a liver graft with APF was ruled out by early initial radiological evaluation. The roles of arterial stenosis, prolonged sepsis, and liver biopsies were questionable, but we considered that development of multiple diffuse APF was of unknown cause. Morphological presentation was that of congenital APF (type 3), which are diffuse and intrahepatic and which can be the most difficult to manage.2

Only about 20 cases of APF after LT have been reported, all secondary to an interventional radio­logical procedure; the presence of multiple intrahepatic APF is very rare. Only Puri and colleagues reported a case quite similar to ours of multiple bilobar APF recognized 1 year after transplant.4 The etiology was considered not clear. The patient was managed conservatively and has not required intervention. In the case of significant portal hypertension secondary to APF, percutaneous transarterial embolization has been increasingly performed in place of surgery, including, rarely, for LT recipients.1,5,6

In conclusion, multiple diffuse hepatic APF can develop after LT and can be hemodynamically significant, causing severe portal hypertension and requiring embolization.


  1. Heaton ND, Davenport M, Karani J, Mowat AP, Howard ER. Congenital hepatoportal arteriovenous fistula. Surgery. 1995;117(2):170-174. doi:10.1016/s0039-6060(05)80081-9
  2. Guzman EA, McCahill LE, Rogers FB. Arterioportal fistulas: introduction of a novel classification with therapeutic implications. J Gastrointest Surg. 2006;10(4):543-550. doi:10.1016/j.gassur.2005.06.022
  3. Saad W, Lippert A, Davies M, et al. Prevalence, presentation, and endovascular management of hemodynamically or clinically significant arterio-portal fistulae in living and cadaveric donor liver transplant recipients. Clin Transplant. 2012;26(4):532-538. doi:10.1111/j.1399-0012.2011.01547.x
  4. Puri Y, Srinivasan P, Peddu P, Heaton N. Case report of multiple bilobar hepatic arterio-portal fistulas post-liver transplantation managed conservatively. Transplant Proc. 2018;50(3):920-924. doi:10.1016/j.transproceed.2017.12.001
  5. Detry O, De Roover A, Delwaide J, Dondelinger RF, Meurisse M, Honore P. Selective coil occlusion of a large arterioportal fistula in a liver graft. Liver Transpl. 2006;12(5):888-889. doi:10.1002/lt.20695
  6. Roy A, Taneja S, Behera A, et al. Intrahepatic arterioportal fistula: a rare cause of portal hypertension after deceased donor liver transplant. Exp Clin Transplant. 2020;18(5):645-648. doi:10.6002/ect.2020.0119

Volume : 19
Issue : 10
Pages : 1114 - 1115
DOI : 10.6002/ect.2021.0199

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From the 1Hospices Civils de Lyon, Edouard Herriot Hospital, Department of Digestive Diseases; the 2Claude Bernard Lyon 1 University; the 3Hospices Civils de Lyon, Croix-Rousse Hospital, Department of Hepatology; and the 4Hospices Civils de Lyon, Edouard Herriot Hospital, Department of Radiology, Lyon, France
Acknowledgements: The authors have not received any funding or grants in support of the presented research or for the preparation of this work and have no declarations of potential conflicts of interest.
Corresponding author: Jérôme Dumortier, pavillon L, Hôpital Edouard Herriot, 69437 Lyon Cedex 03, France
Phone: +33 4 72 11 01 11