Alvarez Alfonso E, Ribeiro Antônio F, Hessel Gabriel, Baracat Jamal, Ribeiro José D
Division of Pediatric Immunology, Allergy, and Pneumology, Department of Pediatrics, State University of Campinas Medical School, Campinas, Brazil.
Pediatr Pulmonol. 2002 Nov;34(5):391-4. doi: 10.1002/ppul.10182.
Hepatopulmonary syndrome (HPS) is the clinical relationship between hepatic disease and the existence of pulmonary vascular dilatations, which can result in a range of arterial oxygenation abnormalities. It is probably caused by an alteration in the synthesis or metabolism of vasoactive pulmonary substances at a hepatic level, leading to vasodilatation of pulmonary vessels and diffusion perfusion defects. The Abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end-to-side or side-to-side shunt. Here, we report on a 5-year-and-11-month-old-boy who had started cyanosis at age 4 years and 11 months, and did not have any other pulmonary or cardiac signs or symptoms. In the investigation, arterial blood gases revealed a PaO(2) of 41.4 mm Hg. The chest x-ray film and echo Doppler cardiography were normal. Nuclear scanning with Technetium 99m-labeled macroaggregated albumin showed the presence of arteriovenous shunt, at 47%. Abdominal echography revealed Abernethy malformation with an absence of portal vein. We concluded that the patient had HPS caused by Abernethy malformation. The possible mechanism is that in this malformation, there is a deviation in the blood that comes from the spleen to the vena cava without passing through the liver, so there is no metabolism of some substances which can be responsible for the imbalance between the vasodilatation and the vasoconstriction of the pulmonary circulation. Abernethy malformation must be included as one of the etiologies of hepatopulmonary syndrome. This is the first case described in the literature with this form of presentation.
肝肺综合征（HPS）是肝脏疾病与肺血管扩张之间的临床关联，可导致一系列动脉氧合异常。它可能是由于肝脏水平上血管活性肺物质的合成或代谢改变，导致肺血管扩张和弥散灌注缺陷。阿伯内西畸形的特征是门静脉血先天性分流，可通过端侧或侧侧分流绕过肝脏。在此，我们报告一名5岁11个月大的男孩，他在4岁11个月时开始出现发绀，且没有任何其他肺部或心脏体征或症状。在检查中，动脉血气显示动脉血氧分压（PaO₂）为41.4 mmHg。胸部X光片和超声心动图检查均正常。用99m锝标记的大聚合白蛋白进行核扫描显示存在动静脉分流，分流率为47%。腹部超声检查显示阿伯内西畸形且无门静脉。我们得出结论，该患者患有由阿伯内西畸形引起的肝肺综合征。可能的机制是，在这种畸形中，来自脾脏的血液未经肝脏而直接分流到腔静脉，因此一些可导致肺循环血管舒张和血管收缩失衡的物质无法进行代谢。阿伯内西畸形必须被列为肝肺综合征的病因之一。这是文献中描述的首例这种表现形式的病例。
