Abstract: This article reported two rare cases of different types of neonatal congenital hepatic arteriovenous fistula (HAVF). One case was vascular HAVF, and abdominal vascular abnormalities of the fetus could not be found before birth, and cardiogenic shock and pulmonary hypertension crisis occurred 28 hours after birth. After continuous follow-up, he developed symptoms of heart failure and pulmonary hypertension 2 days after birth. Another fetus had tumorous HAVF. Both children were diagnosed by abdominal vascular color Doppler ultrasonography and abdominal enhanced CT scan, and were given inotropic diuresis and oral propranolol for symptomatic treatment. After 1 week, the pulmonary arterial pressure gradually decreased, and they were discharged after 2 to 3 week medical cares. During the follow-up of more than 10 months, the imaging examination showed that the HAVF of the two children gradually disappeared, the hemangioma shrank, the growth and development were normal, and no adverse events occurred. At the discussion of this article, the pathophysiology, clinical features, diagnostic methods and treatment options in fetus with congenital HAVF were given at base of literature review, and we hope it might improve neonatologists' awareness of the disease.

Key words: Congenital arteriovenous fistula, Liver, Diagnosis and treatment, Neonate