European Respiratory Journal ( IF 24.3 ) Pub Date : 2022-08-04 , DOI: 10.1183/13993003.00107-2022 Marie Caroline Certain 1, 2, 3 , Audrey Baron 1, 2, 3 , Matthieu Turpin 1, 2, 3 , Nathan Ebstein 1, 2, 3 , Athénaïs Boucly 1, 2, 3 , Antoine Beurnier 1, 2, 3 , Mitja Jevnikar 1, 2, 3 , Anne Roche 1, 2, 3 , Sophia Keddache 1, 2, 3 , Sophie Bulifon 1, 2, 3 , Andrei Seferian 1, 2, 3 , Xavier Jaïs 1, 2, 3 , David Montani 1, 2, 3 , Marc Humbert 1, 2, 3 , Olivier Sitbon 1, 2, 3 , Laurent Savale 2, 3, 4
Portopulmonary hypertension (PoPH) is a subtype of pulmonary arterial hypertension (PAH) complicating the course of portal hypertension [1, 2]. Similar to other causes of PAH, PoPH is characterised by a progressive structural and functional remodelling of small pulmonary arteries leading to pre-capillary pulmonary hypertension defined by a mean pulmonary artery pressure (mPAP) ≥25 mmHg, normal pulmonary artery wedge pressure (PAWP) ≤15 mmHg and raised pulmonary vascular resistance (PVR) >3 Wood Units (WU) [
中文翻译:
毛细血管前肺动脉高压和肺血管阻力在 2 到 3 个伍德单位之间的肝硬化患者的结局
门静脉高压症 (PoPH) 是肺动脉高压 (PAH) 的一种亚型,使门静脉高压症的病程复杂化 [1, 2]。与 PAH 的其他原因相似,PoPH 的特征是小肺动脉进行性结构和功能重塑,导致毛细血管前肺动脉高压,定义为平均肺动脉压 (mPAP) ≥25 mmHg,肺动脉楔压正常 (PAWP) ≤15 mmHg 且肺血管阻力 (PVR) 升高 >3 Wood Units (WU) [