Dollinger M M
Klinik für Innere Medizin I, Martin-Luther-Universität Halle-Wittenberg, Halle.
Praxis (Bern 1994). 2006 Oct 4;95(40):1539-42. doi: 10.1024/1661-8157.95.40.1539.
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two distinct pulmonary complications of liver disease, which seem pathogenetically linked to the presence of portal hypertension. The most common presenting symptom of both syndromes is dyspnoea, but HPS is more prevalent in 5-30% of patients. The diagnosis of HPS requires the documentation of arterial hypoxemia and intrapulmonary vascular dilatation with anatomical shunting. In contrast, POPH has a prevalence of 2-16% and is only considered proven, if other causes of the pulmonary hypertension than the high portal pressure are excluded. Moderate to severe HPS or POPH carry a poor prognosis and liver transplantation remains the only curative treatment, although POPH in particular is associated with high perioperative mortality.
肝肺综合征（HPS）和门脉性肺动脉高压（POPH）是肝脏疾病两种不同的肺部并发症，它们在发病机制上似乎与门脉高压的存在有关。这两种综合征最常见的症状都是呼吸困难，但HPS在5%-30%的患者中更为普遍。HPS的诊断需要记录动脉血氧不足以及伴有解剖分流的肺内血管扩张。相比之下，POPH的患病率为2%-16%，只有排除了除高门脉压之外的其他肺动脉高压病因，才能确诊。中度至重度HPS或POPH预后较差，肝移植仍然是唯一的治愈性治疗方法，不过特别是POPH与围手术期高死亡率相关。
