Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant

Yazıcı, Mutlu Uysal; Çil, Barbaros; Bayrakçı, Benan; Sasmaz, Necati; Baysoy, Gökhan; Gürakan, Figen

doi:10.1055/s-0037-1603822

Cited by 3 publications

(1 citation statement)

References 34 publications

(43 reference statements)

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“…This vascular malformation occurs most frequently as hepatic artery portal vein fistula (HAPF) and HAVF, while hepatic porto systemic venous fistula (HPVF) is a less common variant. The main clinical manifestations of HAPF include portal hypertension caused by the direct high-pressure flow of hepatic arterial blood into the portal vein, splenomegaly, esophageal varices and bleeding, and abdominal pain and ascites (6,7). HAVF, on the other hand, is mainly caused by the direct flow of hepatic arterial blood into the hepatic vein via abnormal channels.…”

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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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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Vascular Disorders of the Liver

Bruyne

2021

Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

Angelico

Paolantonio

Paoletti

et al. 2020

WJH

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BACKGROUND Congenital intrahepatic arterioportal fistula (IAPF) is a rare vascular malformation in infants that causes severe portal hypertension (PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children. Furthermore, indications are not defined. CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multi-step endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised. The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascular-surgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported. CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

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Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant

Cited by 3 publications

References 34 publications

Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates

Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates

Vascular Disorders of the Liver

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

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