Krowka M J, Cortese D A
Mayo Clin Proc. 1987 Mar;62(3):164-73. doi: 10.1016/s0025-6196(12)62438-0.
Severe hypoxemia associated with chronic liver disease is an uncommon disorder most likely due to an intrapulmonary vascular abnormality that has characteristics of both ventilation-perfusion mismatching and diffusion limitation. Anatomically, the intrapulmonary vascular abnormalities can occasionally be detected by angiography. Physiologically, the gas exchange abnormalities can be substantiated by contrast-enhanced two-dimensional echocardiography. Although orthodeoxia and platypnea have frequently been found in these patients, echocardiographic data suggest that vascular abnormalities can exist in the absence of orthodeoxia. We describe 11 patients who had severe hypoxemia and chronic liver disease and review their pulmonary angiographic, contrast echocardiographic, and arterial blood gas findings. Among five of these patients who were given almitrine bismesylate, an experimental medication thought to alter ventilation-perfusion relationships in patients with chronic obstructive pulmonary disease, one had improved oxygenation. We recommend that patients with hypoxemia associated with chronic liver disease have detailed studies to rule out reversible forms of hypoxemia and that those with severe hypoxemia undergo testing to determine the existence of intrapulmonary vascular abnormalities, especially if liver transplantation is considered.
与慢性肝病相关的严重低氧血症是一种罕见的病症，很可能是由于一种肺内血管异常所致，这种异常具有通气-灌注不匹配和弥散受限的特征。从解剖学角度来看，肺内血管异常偶尔可通过血管造影检测到。从生理学角度来看，气体交换异常可通过对比增强二维超声心动图得到证实。尽管在这些患者中经常发现直立性低氧血症和平卧呼吸，但超声心动图数据表明，在没有直立性低氧血症的情况下也可能存在血管异常。我们描述了11例患有严重低氧血症和慢性肝病的患者，并回顾了他们的肺血管造影、对比超声心动图和动脉血气检查结果。在这11例患者中，有5例接受了双嘧达莫，这是一种被认为可改变慢性阻塞性肺疾病患者通气-灌注关系的实验性药物，其中1例患者的氧合情况有所改善。我们建议，患有与慢性肝病相关的低氧血症的患者应进行详细检查，以排除可逆性低氧血症的形式，而那些患有严重低氧血症的患者应接受检查，以确定是否存在肺内血管异常，特别是在考虑进行肝移植的情况下。
