Hervé P, Lebrec D, Brenot F, Simonneau G, Humbert M, Sitbon O, Duroux P
Service de Pneumologie et Réanimation, Hôpital Antoine Béclère, Clamart, France.
Eur Respir J. 1998 May;11(5):1153-66. doi: 10.1183/09031936.98.11051153.
The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance is elevated. Since hepatopulmonary syndrome and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the common factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmonary syndrome seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of portopulmonary hypertension. Resolution of hepatopulmonary syndrome is common after liver transplantation, which has an uncertain effect in portopulmonary hypertension. The pathophysiology of both syndromes may involve vasoactive mediators and angiogenic factors.
肝病和门脉高压症中广泛的肺血管疾病谱，涵盖了以肺内血管扩张为特征的肝肺综合征，到肺血管阻力升高的肺动脉高压（门脉性肺动脉高压）。由于在非肝门脉高压患者中也有肝肺综合征和门脉性肺动脉高压的报道，决定其发生的共同因素必定是门脉高压。临床表现差异很大，肝肺综合征有气体交换受损，而门脉性肺动脉高压有血流动力学衰竭。肝肺综合征的严重程度似乎与肝衰竭的严重程度平行，而肝损害与门脉性肺动脉高压的严重程度之间尚未发现简单的关系。肝肺综合征在肝移植后通常会缓解，而对门脉性肺动脉高压的影响尚不确定。这两种综合征的病理生理学可能都涉及血管活性介质和血管生成因子。
