Rodríguez-Roisin R, Agustí A G, Roca J
Thorax. 1992 Nov;47(11):897-902. doi: 10.1136/thx.47.11.897.
On the basis of previous work, our own experience and findings, and the considerations discussed above, we propose a set of four diagnostic criteria for the hepatopulmonary syndrome: 1. presence of chronic hepatic disease (alcoholic, postnecrotic, or primary biliary cirrhosis or active chronic hepatitis)--severe liver dysfunction may not be mandatory; 2. absence of intrinsic cardiopulmonary disease, with normal chest radiograph or with nodular basal shadowing; 3. pulmonary gas exchange abnormalities--an increased alveolar-arterial oxygen gradient (> or = 2.0 kPa) with or without hypoxaemia; 4. the extrapulmonary appearance of intravenous radiolabelled microspheres or a positive contrast enhanced echocardiogram, suggesting intrapulmonary vascular abnormalities. Although these four criteria appear straightforward, there may be other features that are not always present--namely: 1. low transfer factor (diffusing capacity); 2. shortness of breath, with or without platypnoea and orthodeoxia; 3. increased cardiac output and reduced pulmonary vascular pressures; 4. small (or no) increase in pulmonary vascular resistance when the patient is breathing low oxygen mixtures. From the physiological viewpoint, the hepatopulmonary syndrome provides an excellent model for clinical research in the pathophysiology of pulmonary gas exchange. So far it has been possible to show that arterial hypoxaemia in this condition is (1) partitioned into components resulting from VA/Q mismatching, intrapulmonary shunt, and limitations of oxygen diffusion; (2) modulated by the interplay between the intrapulmonary and the extrapulmonary determinants of PaO2, such as cardiac output and minute ventilation; (3) vulnerable to the influence of inadequate pulmonary vascular tone; and (4) resolved when the injured liver is replaced and hepatic function is restored to within normal limits.
基于先前的研究工作、我们自己的经验和发现，以及上述讨论的因素，我们提出了一套针对肝肺综合征的四项诊断标准：1. 存在慢性肝病（酒精性、坏死后性、原发性胆汁性肝硬化或活动性慢性肝炎）——严重肝功能不全并非必需条件；2. 无内在心肺疾病，胸部X线片正常或有结节状基底阴影；3. 肺气体交换异常——肺泡-动脉血氧梯度增加（≥2.0 kPa），伴或不伴有低氧血症；4. 静脉注射放射性微球的肺外表现或超声心动图造影增强阳性，提示肺内血管异常。尽管这四项标准看似简单明了，但可能存在其他并非总是出现的特征，即：1. 低转运因子（弥散能力）；2. 呼吸急促，伴或不伴有平卧呼吸困难和直立性低氧血症；3. 心输出量增加和肺血管压力降低；4. 患者呼吸低氧混合气时肺血管阻力轻度增加（或无增加）。从生理学角度来看，肝肺综合征为肺气体交换病理生理学的临床研究提供了一个绝佳模型。到目前为止，已经能够证明这种情况下的动脉低氧血症是：（1）由通气/血流比值失调、肺内分流和氧扩散受限导致的成分组成；（2）受动脉血氧分压的肺内和肺外决定因素（如心输出量和分钟通气量）之间相互作用的调节；（3）易受肺血管张力不足的影响；（4）在受损肝脏被替换且肝功能恢复到正常范围内时得以缓解。
