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.
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。.
Keywords: Disease attributes; Portal hypertension; Pulmonary hypertension; Targeted drug.