门静脉高压相关性肺动脉高压治疗现状

Current statue of portal hypertension-related pulmonary hypertension

门静脉高压相关性肺动脉高压(PoPH)是门静脉高压的一种严重并发症,常引起肺血管收缩、重构,其特征为毛细血管前肺动脉高压,是动脉性肺动脉高压(PAH)的重要类型之一,PoPH的发生可能与雌激素代谢有关。PoPH患者生存时间短,预后差。目前PoPH治疗手段有限,主要包括原发病治疗、肺动脉高压靶向药物治疗,肝移植可作为一项治疗手段。本文就PoPH的流行病学、发病机制、临床特征、治疗现状作一综述,旨在提高临床医生对PoPH的认识。

Portopulmonary hypertension(PoPH),a serious complication of portal vein hypertension,often causes pulmonary vasoconstriction and remodeling,characterized by precapillary pulmonary hypertension,PoPH is one of the important types of pulmonary arterial hypertension(PAH)and its occurrence may be related to estrogen metabolism.PoPH patients have a short survival time and poor prognosis.Primary disease treatment,targeted drug therapy for pulmonary hypertension and liver transplantation can be used as the treatment methods.In this review,we reviewed the epidemiology,pathogenesis,clinical characteristics and current treatment for PoPH,aiming to raise awareness for PoPH among clinicians.