Hira H S, Kumar Jaya, Tyagi S K, Jain S K
Divisions of Pulmonary Medicine and Gastroenterology, Department of Medicine, Maulana Azad Medical College New Delhi, India.
Indian J Chest Dis Allied Sci. 2003 Jul-Sep;45(3):165-71.
Hepatopulmonary syndrome consists of a triad of hepatic dysfunction and/or portal hypertension, intrapulmonary vascular dilatations and hypoxemia. A study of hepatopulmonary syndrome among patients of cirrhosis of liver and portal hypertension was undertaken.
Thirty patients participated in this study. The diagnosis of cirrhosis of liver was confirmed by liver biopsy. Arterial blood gas analysis, pulmonary function tests, two-dimensional transthoracic air contrast echocardiography were undertaken in all the patients. Those patients in whom contrast echocardiogram showed intrapulmonary vascular dilatations were classified as the positive group while others were labelled as the negative group.
Ten patients (33.33%) had a positive contrast echocardiogram; five (16.67%) of them were found to have PaO2<70 mmHg and were qualified for the diagnosis of hepatopulmonary syndrome (HPS); and other five (16.67%) with PaO2>70 mmHg were diagnosed as intrapulmonary dilatations syndrome (IPVDS). Five patients of HPS revealed significant P(A-a)O2 gradient and intrapulmonary shunts of moderate severity computed by a/A ratio. Cyanosis (p=0.001), clubbing (p=0.009) and orthodeoxia (p=0.0024) were significantly commoner in the five patients of hepatopulmonary syndrome. Presence of spider naevi was significantly related with the presence of intrapulmonary vascular dilatations.
The study results showed presence of hepatopulmonary syndrome and intrapulmonary vascular dilatation syndrome among patients of portal hypertension. The presence of cyanosis, clubbing and orthodeoxia were found to be suggestive indicators of hepatopulmonary syndrome. Even though not very specific, spider naevi were found to be a useful clinical indicator for the presence of intrapulmonary vascular dilatations.
肝肺综合征由肝功能不全和/或门静脉高压、肺内血管扩张和低氧血症三联征组成。对肝硬化和门静脉高压患者的肝肺综合征进行了一项研究。
30名患者参与了本研究。肝活检证实为肝硬化。对所有患者进行动脉血气分析、肺功能测试、二维经胸空气对比超声心动图检查。对比超声心动图显示肺内血管扩张的患者分为阳性组，其他患者标记为阴性组。
10名患者（33.33%）对比超声心动图呈阳性；其中5名（16.67%）患者动脉血氧分压（PaO2）<70 mmHg，符合肝肺综合征（HPS）诊断标准；另外5名（16.67%）患者PaO2>70 mmHg，诊断为肺内扩张综合征（IPVDS）。5例HPS患者显示显著的肺泡-动脉血氧分压差（P(A-a)O2）梯度以及通过a/A比值计算的中度严重程度的肺内分流。在5例肝肺综合征患者中，发绀（p=0.001）、杵状指（p=0.009）和直立性低氧血症（p=0.0024）明显更为常见。蜘蛛痣的存在与肺内血管扩张的存在显著相关。
研究结果表明门静脉高压患者中存在肝肺综合征和肺内血管扩张综合征。发现发绀、杵状指和直立性低氧血症是肝肺综合征的提示性指标。蜘蛛痣虽然不是非常特异，但被发现是肺内血管扩张存在的有用临床指标。
