门脉高压性肺动脉高压临床特点及靶向药物治疗分析

Analysis of clinical features and targeted drug therapy of portopulmonary hypertension

目的分析门脉高压性肺动脉高压(PoPH)的临床特点及靶向药物治疗效果.方法连续纳入2017年1月1日至2018年12月31日于北京朝阳医院呼吸与危重症医学科住院的PoPH患者共5例,收集患者临床和随访资料,对患者病史、临床表现、右心导管检查(RHC)、心功能及肝功能分级、具体治疗方案和预后进行分析.结果5例PoPH患者中,男3例,女2例,确诊中位年龄为56岁.门脉高压的基础病均为肝硬化,1例患者同时存在肝肺综合征(HPS).5例患者均以活动后呼吸困难为主要呼吸系统症状,从症状出现到就诊的中位时间为1年(5个月~8年).所有患者均以RHC作为肺动脉高压的确诊标准,肺动脉平均压中位数42 mmHg(1 mmHg=0.133 kPa),肺血管阻力中位数为538 dyn·s·cm^-5.Child-Pugh肝功能分级3例B级,2例A级,与心功能不全的严重程度不相匹配.1例患者行肝移植术,术后持续右心功能衰竭,应用靶向药物治疗后缓解.5例患者均应用肺动脉高压靶向药物治疗.规律应用肺动脉高压靶向药物且按时随访的3例患者,随访期间心功能指标有不同程度改善.2例未规律用药、随访的患者,心功能无改善甚至恶化.1例患者死亡,为肝移植术后的患者,死亡原因为重症肺炎、右心功能衰竭,移植后生存期1年.结论PoPH患者的肝脏储备功能与心功能分级不相匹配,PoPH可与HPS同时存在.规律应用肺动脉高压靶向药物可能使PoPH患者获益.

Objective To analyze the clinical characteristics and the effect of targeted drug therapy of portopulmonary hypertension(PoPH).Methods A total of 5 patients with PoPH who were admitted to the Department of Pulmonary and Critical Care Medicine of Beijing Chao-Yang Hospital from January 1,2017 to December 31,2018 were included.The clinical information and follow-up data were collected.The patient′s medical history,clinical manifestations,right cardiac catheterization(RHC),classification of cardiac and hepatic function,treatment and prognosis were analyzed.Results Among the 5 patients with PoPH,3 were male and 2 were female.The median age was 56 years.The underlying diseases of portal hypertension were all cirrhosis,and 1 patient combined with hepatopulmonary syndrome(HPS).Dyspnea was the main respiratory symptom in all the 5 patients,and the median time from symptom onset to diagnosis was 1 year(5 months to 8 years).RHC was used as the diagnostic criteria for pulmonary hypertension in all patients,with a median mean pulmonary arterial pressure of 42 mmHg(1 mmHg=0.133 kPa)and a median pulmonary vascular resistance of 538 dyn·s·cm^-5.3 cases were in Child-Pugh liver function grade B,and 2 were in grade A.The hepatic reserve function was not matched with the severity of cardiac insufficiency.Liver transplantation was performed in 1 patient,whose right ventricular dysfunction can be alleviated by targeted drug therapy after operation.All the 5 patients received targeted drug therapy of pulmonary hypertension.In the 3 patients who were regularly treated with targeted drugs and followed up on time,the cardiac function was improved during the follow-up period.There was no improvement or even deterioration of cardiac function in 2 patients who were not regularly treated or followed up.One patient died after liver transplantation.The cause of death was severe pneumonia and right ventricular dysfunction.The survival time after transplantation was 1 year.Conclusions In PoPH patients,the hepatic reserve function is not matched with the heart function classification.PoPH can coexist with HPS.Regular application of pulmonary hypertension targeting drugs may benefit patients with PoPH.