目的 探讨新生儿期起病的肝血管瘤伴动静脉瘘（HH-AVF）的临床特征、治疗及转归情况。方法 收集2018年1月至2024年12月入院的8例新生儿HH-AVF的临床资料，分析其临床特点，评价介入治疗效果。结果 8例HH-AVF患儿，男5例、女3例，均存在肝脏占位病变。6例患儿均有呼吸困难需呼吸机支持，胸部X线提示心影增大，并发心力衰竭、肺动脉高压；2例患儿有皮肤多发血管瘤；1例有颈部血管瘤并伴出血。8例HH-AVF患儿均接受介入治疗，介入治疗时日龄（29.5±9.5） d，同时口服普萘洛尔，1例术后4个月复诊超声提示肝脏肿物增大，考虑瘤体进展进行二次介入栓塞治疗，其余7例好转出院。随访12~24个月，所有患儿均未再出现呼吸困难、肺动脉高压及心力衰竭等表现，且病灶均较确诊时明显减小或消失。结论 新生儿期起病的HH-AVF临床表现多样，伴心力衰竭、肺动脉高压的患儿病死率较高。介入治疗可能是改善HH-AVF预后的有效手段。

Objective To investigate the clinical features, therapeutic strategies, and prognostic outcomes of hepatic hemangioma with arteriovenous fistula (HH-AVF) in neonates. Methods Clinical data of 8 neonates diagnosed with HH-AVF and admitted to our institution between January 2018 and December 2024 were retrospectively collected. Their clinical manifestations were analyzed, with the efficacy of interventional therapy evaluated. Results Among the 8 neonates with HH-AVF (5 males and 3 females), all presented with hepatic space-occupying lesions. Six cases developed respiratory distress requiring mechanical ventilation, with chest radiography revealing cardiomegaly and complications including heart failure and pulmonary hypertension. Two cases had multiple cutaneous hemangiomas, and one case had a cervical hemangioma with bleeding. All 8 neonates underwent interventional therapy at a median age of (29.5±9.5) days, combined with oral propranolol. One neonate showed an increase in hepatic mass on follow-up ultrasound at 4 months post-operation, prompting a second interventional embolization due to suspected tumor progression; the remaining 7 cases improved and were discharged. During a follow-up period of 12-24 months, none of the patients experienced recurrence of respiratory distress, pulmonary hypertension, or heart failure, and all lesions were significantly reduced or resolved compared to the time of diagnosis. Conclusions Neonatal-onset HH-AVF exhibits diverse clinical manifestations, with higher mortality rates in cases complicated by heart failure and pulmonary hypertension. Interventional therapy may be an effective approach to improve the prognosis of HH-AVF.