van Hirtum P V, Bootsma G P, de Man R A, van Deursen C Th B M, Bonta P I, de Kruif M D
Universitair Medisch Centrum Utrecht, afd. Longziekten, Utrecht.
Ned Tijdschr Geneeskd. 2017;161:D1387.
Hepatopulmonary syndrome is a severe complication of liver disease, with greatly increased mortality. The syndrome is characterized by increased blood-flow, intrapulmonary vasodilatation and angiogenesis, leading to effects including the formation of shunts. This leads to a decrease in arterial oxygen pressure. Liver transplantation is the only effective treatment.
A 74-year-old woman with cirrhosis of the liver attended the pulmonary outpatients' clinic with progressive dyspnoea, which worsened if she sat upright from a lying position (platypnoea). Contrast echocardiography confirmed the diagnosis 'hepatopulmonary syndrome'. The patient was not eligible for liver transplantation. She was given oxygen therapy and died from decompensated cirrhosis of the liver eighteen months later.
Early recognition of hepatopulmonary syndrome is important, because patients may be given priority for liver transplantation. Contrast echocardiography is indicated in patients with liver disease and suffering from hypoxaemia for which there is no other explanation, to reveal the presence of intrapulmonary shunt.
肝肺综合征是肝脏疾病的一种严重并发症，死亡率大幅增加。该综合征的特征是血流增加、肺内血管扩张和血管生成，导致包括分流形成在内的一系列影响。这会导致动脉血氧分压降低。肝移植是唯一有效的治疗方法。
一名74岁肝硬化女性因进行性呼吸困难就诊于肺科门诊，若从卧位坐起（端坐呼吸），呼吸困难会加重。对比超声心动图确诊为“肝肺综合征”。该患者不符合肝移植条件。给予其氧疗，18个月后死于失代偿期肝硬化。
早期识别肝肺综合征很重要，因为患者可能会因此获得肝移植优先权。对于患有肝病且存在无法用其他原因解释的低氧血症患者，建议进行对比超声心动图检查，以揭示肺内分流的存在。
