Mal H, Burgière O, Durand F, Fartoukh M, Cohen-Solal A, Fournier M
Service de Pneumologie et Réanimation Respiratoire, Hopital Beaujon, Clichy, France.
J Hepatol. 1999 Aug;31(2):360-4. doi: 10.1016/s0168-8278(99)80236-0.
We report the case of a patient with liver cirrhosis who successively developed hepatopulmonary syndrome and portopulmonary hypertension. Initially, the patient presented with severe dyspnea and hypoxemia at rest. Technetium-99 macroaggregated albumin lung perfusion scan demonstrated right-to-left shunt, and hemodynamic study revealed a hyperdynamic state with low pulmonary vascular resistance, thus confirming the diagnosis of hepatopulmonary syndrome. More than 2 years after the onset of pulmonary symptoms, a marked improvement in dyspnea and gas exchange was observed. Lung perfusion scan did not disclose any right-to-left shunt and right-sided heart catheterization showed evidence of severe pulmonary hypertension. We conclude that hepatopulmonary syndrome and portopulmonary hypertension are not mutually exclusive. We hypothesize that, by reversing the phenomenon of intrapulmonary vasodilatation, the development of portopulmonary hypertension interfered with each of the potential causes of hypoxemia in hepatopulmonary syndrome (ventilation-perfusion inequalities, intrapulmonary shunting, oxygen diffusion limitation) and, as a result, led to a correction of hypoxemia.
我们报告了一例肝硬化患者，该患者先后出现肝肺综合征和门肺高压。最初，患者在静息时出现严重呼吸困难和低氧血症。锝-99 大聚合白蛋白肺灌注扫描显示存在右向左分流，血流动力学研究显示为高动力状态且肺血管阻力降低，从而确诊为肝肺综合征。肺部症状出现 2 年多后，观察到呼吸困难和气体交换有明显改善。肺灌注扫描未发现任何右向左分流，右侧心导管检查显示有严重肺动脉高压的证据。我们得出结论，肝肺综合征和门肺高压并非相互排斥。我们推测，通过逆转肺内血管扩张现象，门肺高压的发展干扰了肝肺综合征中低氧血症的每一个潜在原因（通气-灌注不均、肺内分流、氧弥散受限），结果导致低氧血症得到纠正。
