门脉高压相关性肺动脉高压临床分析

目的探讨门脉高压相关性肺动脉高压(PoPH)的临床特点和经胸超声心动测量右心房线性指数同心功能或肝功能的关系。 方法选择首都医科大学附属北京安贞医院呼吸与危重症医学科2012年1月至2018年12月收治的门脉高压相关性肺动脉高压患者11例，回顾性分析11例患者的临床症状和体征、影像检查资料、实验室检查结果、右心导管检查测算数据、心肺功能测定结果、治疗和转归等。所有患者进行NHYA分级与mMRC量表评分。实验室检查结果包括血常规、生化、凝血、B型脑钠肽、动脉血气分析等，用以计算Child-Pugh及MELD等评分。 结果11例中男性5例，女性6例，肝硬化门脉高压者9例，非肝硬化门脉高压者2例，追溯病史发现肺动脉高压距离门脉高压诊断时间为0.5至19年，中位时间5年。NHYA分级与改良MRC呼吸困难(mMRC)评分存在正相关关系r＝0.9608，95%CI(0.8460，0.9896), P<0.0001]，与Child-Pugh评分无相关关系。右心房线性指数与BNP存在相关关系r＝0.7930，95%CI(0.3684，0.9439)，P＝0.0036]，与终末期肝脏疾病评分(MELD)无相关关系。所有患者均存在不同程度的低氧血症，肺泡-动脉血氧分压差值较预计值升高(t＝3.669，P＝0.0018)，具有显著性差异。就诊时已出现右心功能严重下降的患者预后差。 结论PoPH患者多以运动性呼吸困难为首发症状，与心功能严重程度相关，与肝功能无显著线性关系，临床随访不能只评价肝功能。随访中超声心动图中右心房径线的测量有助于心功能评估。尽早干预肺动脉压力的升高，有助于右心功能的保护，改善预后。

Clinical analysis of portopulmonary hypertension

Objective To explore the clinical features of portopulmonary hypertension(PoPH)and the relationship between the right atrial liner index and the heart function.Methods Eleven patients were diagnosed as PoPH in Beijing Anzhen Hospital,Capital Medical University in China from January 2012 to December 2018.The general information,clinical manifestation,medical imaging results,laboratory tests,right heart catheter data,cardiopulmonary exercise test,treatment and prognosis of the patients were analyzed retrospectively.The laboratory tests included the complete blood count,serum chemistry profile,coagulation function,B type brain natriuretic peptide,and arterial blood gas analysis,which were used to calculate the scales of Child-Pugh and Model for the end-stage liver disease(MELD).Results The patients(5 males and 6 females)aged from 29 to 85 years.Hepatocirrhosis was found in 9 cases and non-hepatocirrhosis was found in 2 cases.According to the medical history,the onset time of pulmonary hypertension was 0.5-19 years after portal vein hypertension,with the median time of 5 years.The scale of New York Heart Association(NYHA)was positively correlated with the modified Medical Research Council(mMRC)scaler=0.9608,95%CI(0.8460,0.9896),P<0.0001],however,it had no correlation with Child-Pugh scale.The correlation coefficient was 0.7930 between the right atrial linear index and the BNP with the 95%CI of 0.3684-0.9439(P=0.0036).However,no significant difference was found between the right atrial linear index and the MELD.All the patients suffered from hypoxemia.The alveolar-arterial oxygen differential pressure(PA-aO 2)was higher significantly than the predicted value(t=3.669,P=0.0018).The patients with severe heart failure had poor prognosis.Conclusion The patients with PoPH are more likely to experience exertional dyspnea,which is associated with the degree of the heart dysfunction.And it is a fact that all the patients have hypoxemia.Therefore,the clinical decision for the patients with PoPH should not be made only in the consideration of the liver function.The right atrial linear index is an available option to follow such patients.