婴儿期先天性门腔静脉分流导致肺内血管扩张性低氧血症：病例报告。
Hypoxia due to intrapulmonary vascular dilatation in a toddler with a congenital portacaval shunt: case report.
机构信息
Departments of Pediatrics at Tawam Hospital, Alain City, United Arab Emirates.
KidsHeart Medical Center, Alain City, United Arab Emirates.
出版信息
BMC Pulm Med. 2019 Feb 22;19(1):49. doi: 10.1186/s12890-019-0788-8.
BACKGROUND
The term hepatopulmonary syndrome typically applies to cyanosis that results from "intrapulmonary vascular dilatation" due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen.
CASE PRESENTATION
Here, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. A transcatheter embolization of the shunt resulted in resolution of his findings.
CONCLUSIONS
Congenital portosystemic shunts need to be considered in the differential diagnosis of cyanosis.
背景
肝肺综合征一词通常适用于由于晚期肝病导致的“肺内血管扩张”引起的发绀。类似的发现也可能是由于先天性门体分流而没有肝病引起的。这种分流的一个不良后果是肺内血管扩张，这会影响氧气的微血管气体交换单位。
病例介绍
在这里，我们描述了一个患有慢性发绀、运动不耐受和杵状指的幼儿，其病因是门静脉和下腔静脉之间的畸形分流。分流的经导管栓塞导致其症状缓解。
结论
在发绀的鉴别诊断中需要考虑先天性门体分流。
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