新生儿先天性肝血管瘤伴动静脉瘘导致心功能不全1例

Heart failure caused by congenital hepatic hemangioma complicated with arteriovenous fistula in a neonate

摘要:

患儿女，3日龄，因生后气促3 d入院。患儿为胎龄36⁺²周早产儿。产前胎儿超声提示胎儿肝血管发育异常，生后即出现呼吸急促，需要呼吸支持。胸部X线检查提示心影增大，心脏彩超提示右心房和右心室增大，肝脏超声、腹部增强CT明确诊断为肝血管瘤伴动静脉瘘。19日龄时在全身麻醉下行肝动脉结扎术，术后心功能好转出院。基因检测显示ACVRL1基因变异，该变异来自患儿母亲。该文主要介绍1例由肝血管瘤伴肝动静脉瘘导致新生儿心功能不全病例，以及对该疾病的多学科诊疗。

Abstract:

The three-day-old female infant was admitted to the hospital due to respiratory distress after birth. She was born premature at 36⁺² weeks gestational age. Prenatal ultrasound suggested abnormal development of the fetal liver vessels, and she had dyspnea that required respiratory support after birth. Chest X-ray indicated an enlarged cardiac silhouette, and cardiac ultrasound revealed enlargement of the right atrium and right ventricle. Diagnosis of hepatic hemangioma with arteriovenous fistula was confirmed through liver ultrasound and abdominal enhanced CT. At 19 days old, she underwent ligation of the hepatic artery under general anesthesia, which led to an improvement in cardiac function and she was subsequently discharged. Genetic testing revealed a mutation in the ACVRL1 gene, which was inherited from the mother. The article primarily introduces a case of neonatal heart failure caused by hepatic hemangioma with arteriovenous fistula, and multi-disciplinary diagnosis and treatment of this disease.