Portal hypertension induced by congenital hepatic arterioportal fistula: Report of four clinical cases and review of the literature

Zhang, Danying; Weng, Shuqiang; Dong, Ling; Shen, Xin; Qu, Xu-Dong

doi:10.3748/wjg.v21.i7.2229

Cited by 38 publications

(34 citation statements)

References 46 publications

(45 reference statements)

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“…It is well known that lasting AP fistula develops portal hypertension and causes several typical hepatic symptoms such as ascites and varices [16, 17]. Fig 5 indicates that lasting AP fistula was strongly correlated with a poor prognosis compared with transient AP fistula.…”

Section: Discussionmentioning

confidence: 94%

Impact of Radiofrequency Ablation-Induced Glisson’s Capsule-Associated Complications in Patients with Hepatocellular Carcinoma

et al. 2017

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BackgroundRadiofrequency ablation (RFA) is commonly used to locally treat hepatocellular carcinoma (HCC). However, when tumors are close to the Glisson’s capsule, RFA may induce injury in this region, complicating therapeutic efforts. We investigated the impact of RFA-induced Glisson’s capsule-associated complications on liver function and prognosis of HCC patients.MethodsWe retrospectively reviewed our patient database and found 170 early-stage HCC patients treated via RFA from April 2004 to December 2012. We defined RFA-induced Glisson’s capsule-associated complication as lasting hepatic arterioportal (AP) fistula, major intrahepatic bile-duct dilatation (affecting two or more subsegments), or hepatic infarction. We also defined liver failure as initial occurrence of either total bilirubin increase (>3.0 mg/dL), uncontrolled ascites, or encephalopathy.ResultsIn our cohort, 15 patients had RFA-induced Glisson’s capsule-associated complications (incidence of related complications, with some overlap: lasting AP fistula, n = 9; major intrahepatic bile-duct dilatation, n = 7; and hepatic infarction, n = 2). The cumulative incidence of liver failure before stage progression was significantly higher and the median overall survival (OS) was significantly lower (52.3 months) in HCC patients with Glisson’s capsule-associated complications than in those without Glisson’s capsule-associated complications (95.0 months). In addition, multivariate analysis demonstrated that Glisson’s capsule-associated complication was a significant independent factor associated with OS.ConclusionsIn this study, we have shown that early-stage HCC patients with RFA-induced Glisson’s capsule-associated complications may have higher risks in poor prognosis.

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Section: Discussionmentioning

confidence: 94%

Impact of Radiofrequency Ablation-Induced Glisson’s Capsule-Associated Complications in Patients with Hepatocellular Carcinoma

et al. 2017

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“…This blood then returns to the heart via the inferior vena cava, leading to the aggravation of the pre-cardiac load. The early signs of HAVF include compensable heart enlargement, increased cardiac output and arrhythmia, and late-onset heart failure (8).…”

Section: Discussionmentioning

confidence: 99%

Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates

Peng

et al. 2020

Front. Pediatr.

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Introduction: Hepatic arteriovenous fistula (HAVF) is an abnormal communication between the hepatic arteries and hepatic veins. This condition is treated mainly using interventional closure and surgery. However, these procedures are associated with many postoperative complications and high mortality. Propranolol and other beta blockers have been used widely and effectively to treat infantile hemangiomas. However, no reports describe the use of these drugs to treat congenital HAVF.Case Description: Here, we present two cases in which beta blocker therapy was used to treat congenital HAVF in neonates. In both cases, antenatal examinations revealed cardiac enlargement and hepatic space-occupying lesions. After birth, both patients rapidly presented with respiratory distress, cyanosis, and heart failure. Echocardiography suggested enlargement of the right heart, widening of the pulmonary artery, and severe pulmonary arterial hypertension, and hepatic examinations revealed HAVF.Results: After admission, the patients were treated with dopamine, milinone, and furosemide for heart failure. However, their conditions worsened, as indicated by nodlike breathing and cyanosis. Endotracheal intubation and ventilator-assisted breathing and a small dose of oral propranolol (1 mg/kg/d) were initiated. The patients' conditions improved, as indicated by decreases in levels of the N-terminal pro-hormone BNP, and the ventilators were removed. The propranolol dose was increased gradually to 2 mg/kg/d. After 2 weeks of propranolol treatment, the neonate in case 2 developed bronchospasm, which improved after propranolol treatment ended and metoprolol treatment was initiated. Liver imaging performed 8-9 months after beta blocker therapy suggested the disappearance of the arteriovenous fistulae in case 2, and close to disappearing of the arteriovenous fistulae in case 1.Conclusion: Propranolol and metoprolol can effectively treat HAVF in infants, an observation consistent with that found in earlier studies that have shown beta blockers are a valid medical treatment option for infantile hemangioma. However, future studies should explore the underlying potential mechanism.

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“…The reviews of congenital IAPF reported that congenital IAPF mostly occurred in infants or young children, but clinical symptoms, which were influenced as consequences of imbalances in Starling's law, depended on the type, size, and number of shunts. 9 Small IAPF can be asymptomatic, but larger IAPF can provoke the symptoms of portal hypertension, which may lead to ascites, variceal hemorrhage, and hepatic encephalopathy. The severity of portal hypertension relies on the location of the fistula, amount of blood flow, and resistance of the liver parenchyma.…”

Section: Discussionmentioning

confidence: 99%

“…11 On CT and magnetic resonance imaging, IAPF may be indicated by earlier enhancement of the portal vein in the arterial phase and earlier enhancement of the portal vein branches than that of the main portal vein. 2,9,21 Intrahepatic arterioportal fistula is detected as an arterial stain lesion accompanied by chronic liver disease, such as hepatocellular carcinoma; thus, the differential diagnosis of IAPF is important. The treatment of IAPF traditionally consists of IVR or surgery.…”

Section: Discussionmentioning

confidence: 99%

A successful case of deceased donor liver transplantation for a patient with intrahepatic arterioportal fistula

Takagi

Yagi

Yoshida

et al. 2016

Hepatology Research

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Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension that is often difficult to treat with interventional radiology or surgery. Liver transplantation for IAPF is extremely rare. We report a case of bilateral diffuse IAPF with severe portal hypertension requiring deceased donor liver transplantation (DDLT). A 51-year-old woman with no past medical history was admitted to another hospital complaining of abdominal distension and marasmus. A computed tomography scan and digital subtraction angiography indicated a massive pleural effusion, ascites, and a very large IAPF. Several attempts of interventional embolization of the feeding artery failed to ameliorate arterioportal shunt flow. As ruptures of the esophageal varices became more frequent, hepatic encephalopathy worsened. After repeated, uncontrollable attacks of hepatic coma, the patient was referred to our facility for further treatment. Surgical approaches to IAPF other than liver transplantation were challenging because of diffuse collateralization; therefore, we placed the patient on the national waiting list for DDLT. Although her Model for EndStage Liver Disease score was relatively low, she received a DDLT 2 months after the waiting period. The postoperative course was uneventful, and the patient was discharged 44 days after her transplant. Liver transplantation may be a valid treatment option for uncontrollable IAPF with severe portal hypertension.

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Portal hypertension induced by congenital hepatic arterioportal fistula: Report of four clinical cases and review of the literature

Cited by 38 publications

References 46 publications

Impact of Radiofrequency Ablation-Induced Glisson’s Capsule-Associated Complications in Patients with Hepatocellular Carcinoma

Impact of Radiofrequency Ablation-Induced Glisson’s Capsule-Associated Complications in Patients with Hepatocellular Carcinoma

Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates

A successful case of deceased donor liver transplantation for a patient with intrahepatic arterioportal fistula

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