中国肺高血压诊断和治疗指南2018

肺动脉高压诊治进展：从指南到实践（2020完整版）1995年治疗肺动脉高压第一个靶向药物上市以来，三大途径实际种药物陆续批准应用，肺动脉高压治疗从传统治疗时代进入了靶向药物的治疗时代。

PAH患者的生存率明显提高，患者的生活质量的到大大改善。

但总体预后依然需要进一步提高和改善。

下面我们一起从肺动脉高压定义、诊断、风险评估以及治疗几个方面，从指南到实践，回顾肺动脉高压诊治进展。

1.肺动脉高压定义的更新：肺动脉高压是一种以肺动脉压力升高为表现形式的血流动力学状态。

自1973年召开的第一届世界肺动脉高压大会以来，肺动脉高压血流动力学标准一直被定义为：在海平面，平卧静息状态下，经右心导管测量肺动脉平均压（mean pulmonary artery pressure，mPAP）≥25mmHg。

然而，在正常成人静息状态下，mPAP正常值为14±3.3mmHg，即使考虑年龄、性别、种族等因素，mPAP正常也不超过20mmHg。

可见，在肺动脉高压定义诊断的标准和正常肺动脉压力中间有个一空间值GAP，相当于存在一个灰色地带。

近年来随着国内外专家对肺动脉高压的认识，这个GAP也得到了重视和研究，发现mPAP超过20mmHg即使不足25mmHg者同样是一种肺动脉压力增高的异常状态，当肺动脉20mmHg ≤mPAP≤25mmHg者与肺动脉压力正常者比较，疾病进展及死亡的风险显著增加。

因此认为，20mmHg≤mPAP≤25mmHg提示是肺血管病变的早期阶段，从而在2018年第六届肺动脉高压大会上，专家们提出是否需要将肺动脉高压血流动力学定义修订为mPAP > 20mmHg。

尽管如上所述，在不同的情况下，mPAP>20mmHg与增加死亡风险相关，但是mPAP从25mmHg降低至20mmHg是否就能改善生存率是尚未被证实。

何况肺动脉高压只是一种血流动力学状态，单纯以压力定义肺动脉高压不能反映疾病的本质。

此外，从血流动力学上，肺动脉高压可分为毛细血管前肺动脉高压与毛细血管后肺动脉高压，前者是指存在毛细血管前的肺动脉病变，并由此产生肺动脉高压，后者则主要是指左心疾病患者肺静脉压力增高引起肺动脉压力被动性地增高，这部分患者早期并不存在毛细血管前的肺动脉病变。

间断重复应用左西孟旦治疗肺动脉高压右心衰竭的临床观察作者：韦斌张朝勇邓燕钟诗颖韦哲黄凯来源：《中国药房》2022年第14期中图分类号 R972+.1;R543.2 文献标志码 A 文章编号 1001-0408（2022）14-1754-05DOI 10.6039/j.issn.1001-0408.2022.14.16摘要目的观察间断重复应用左西孟旦治疗肺动脉高压右心衰竭的疗效和安全性。

方法选择2019年1月-2021年5月在广西医科大学第一附属医院住院治疗的70例肺动脉高压右心衰竭患者，按随机数字表法分为对照组和观察组，每组各35例。

对照组中肺动脉高压危险分层为中危的患者给予他达拉非片和安立生坦片，高危患者给予他达拉非片、安立生坦片和曲前列尼尔注射液，同时均给予抗右心衰竭药物;观察组患者在对照组治疗的基础上静脉泵入左西孟旦注射液12.5 mg，每月1次，泵入速度为0.05～0.1 μg/（kg·min），共3个月。

观察两组患者治疗有效率，治疗后肺动脉高压危险分层改善情况，治疗前后肺动脉平均压、6 min步行距离（6MWD）、血清NT-proBNP水平、右心房压（RAP）、混合静脉血氧饱和度（SvO2）、心指数（CI）、右心室射血分数（RVEF）、三尖瓣环舒张早期血流峰速度/三尖瓣环舒张早期运动峰速度（E/Ea）、三尖瓣环收缩期位移（TAPSE）、右心室舒张末期内径（RVEDD）、右心室收缩末期内径（RVESD）以及不良反应发生情况。

结果治疗后，观察组患者的治疗有效率显著高于对照组（P0.05）。

结论间断重复应用左西孟旦可以改善肺动脉高压右心衰竭患者的右心功能，且安全性较好。

关键词左西孟旦;右心衰竭;肺动脉高压;强心剂;间断重复;疗效;安全性Clinical observation of intermittently repeated application of levosimendan in the treatment of right heart failure of pulmonary hypertensionWEI Bin1，ZHANG Chaoyong1，DENG Yan2，ZHONG Shiying1，WEI Zhe1，HUANG Kai1（1. Cardiac Intensive Care Unit， the First Affiliated Hospital of Guangxi Medical University，Nanning 530021， China; 2. Dept. of Ultrasound， the First Affiliated Hospital of Guangxi Medical University， Nanning 530021， China）ABSTRACT OBJECTIVE To observe the efficacy and safety of intermittently repeated application of levosimendan in the treatment of right heart failure of pulmonary hypertension. METHODS A total of 70 cases of right heart failure of pulmonary hypertension were collected from the First Affiliated Hospital of Guangxi Medical University during Jan. 2019-May 2021， and then randomly divided into control group and observation group， with 35 cases in each group.In control group， the patients with moderate risk of classification of pulmonary hypertension were given Tadalafil tablets and Ambrisentan tablets， while the patients with high risk were given Tadalafil tablets， Ambrisentan tablets and Treprostinil injection; both were given anti-right heart failure drugs at the same time. On the basis of treatment in the control group， patients in the observation group were additionally intravenously pumped with levosimendan injection 12.5 mg， once a month， at a rate of 0.05-0.1 μg/ （kg·min）， 3 months in total. Response rate of therapy， improvement of risk stratification of pulmonary hypertension after treatment， and average pulmonary artery pressure，six-minute walk distance （6MWD）， serum level of N-terminal pro brain natriuretic peptide （NT-proBNP），right atrial pressure （RAP），oxygen saturation in mixed venous blood （SvO2）， cardiac index （CI）， right ventricular ejection fraction （RVEF），early diastolic tricuspid inflow velocity/early diastolic tricuspid annular velocity （E/Ea）， tricuspid annular plane systolic excursion （TAPSE），right ventricular end-diastolic diameter （RVEDD）， right ventricular end-systolic diameter （RVESD） before and after treatment， and the occurrence of adverse reactions were observed in 2 groups. RESULTS The total response rate of observation group was significantly higher than control group after treatment （P0.05）. CONCLUSIONS Intermittently repeated application of levosimendan can improve the function of right heart of pulmonary hypertension complicated with right heart failure and has good safety.KEYWORDS levosimendan;right heart failure;pulmonary hypertension;inotropicagent;intermittently repeated; efficacy; safety肺动脉高压是一种以毛细血管前肺血管异常高压为特征的疾病，右心衰竭是肺动脉高压患者的严重并发症之一，肺动脉高压患者一旦进展为右心衰竭，其死亡率会显著升高[1]。

- 19 -①福建医科大学附属泉州第一医院　福建　泉州　362000沙库巴曲缬沙坦钠与雷米普利治疗老年急性失代偿心力衰竭合并肺动脉高压患者的效果比较何雅芬①　曾又晓①　黄雪娥①【摘要】　目的：比较沙库巴曲缬沙坦钠与雷米普利治疗老年急性失代偿心力衰竭（ADHF）合并肺动脉高压（PAH）的效果。

方法：选取2020年11月—2022年11月于福建医科大学附属泉州第一医院就诊的100例ADHF 合并PAH 老年患者作为研究对象，按照随机数表法将患者分为对照组和观察组，各50例。

对照组予以雷米普利治疗，观察组予以沙库巴曲缬沙坦钠治疗。

比较两组治疗效果、治疗前后血清指标和心功能指标、不良反应发生情况。

结果：治疗3个月后，观察组总有效率高于对照组，差异有统计学意义（P <0.05）。

治疗3个月后，两组N 末端B 型利钠肽原（NT-proBNP）、转化生长因子β1（TGF-β1）水平较治疗前降低，6分钟步行试验（6MWT）、心排血量及左心室射血分数（LVEF）水平较治疗前升高，且观察组优于对照组，差异有统计学意义（P <0.05）。

观察组治疗期间不良反应发生率低于对照组，差异有统计学意义（P <0.05）。

结论：沙库巴曲缬沙坦钠治疗老年ADHF 合并PAH 较雷米普利的临床效果显著，可明显提升心脏功能，改善血清相关指标，且安全性良好。

【关键词】　急性失代偿性心力衰竭　肺动脉高压　雷米普利　沙库巴曲缬沙坦钠 doi：10.14033/ki.cfmr.2023.28.005 文献标识码　A 文章编号　1674-6805（2023）28-0019-04 Comparison Effects of Sacubitril Valsartan Sodium and Ramipril in the Treatment of Elderly Patients with Acute Decompensated Heart Failure Complicated with Pulmonary Arterial Hypertension/HE Yafen, ZENG Youxiao, HUANG Xue ’e. //Chinese and Foreign Medical Research, 2023, 21(28): 19-22 [Abstract]　Objective: To compare the effect of Sacubitril Valsartan Sodium and Ramipril in the treatment of elderly patients with acute decompensated heart failure (ADHF) complicated with pulmonary arterial hypertension (PAH). Method: A total of 100 elderly patients with ADHF complicated with PAH who treated in Quanzhou First Hospital Affiliated to Fujian Medical University from November 2020 to November 2022 were selected as the research objects, the patients were divided into the control group and the observation group according to the random number table method, with 50 cases in each group. The control group was treated with Ramipril, and the observation group was treated with Sacubitril Valsartan Sodium. The therapeutic effects, serum indicators and cardiac function indicators before treatment and after 3 months of treatment, and adverse reactions of two groups were compared. Result: After 3 months of treatment, the total effective rate of the observation group was higher than that of the control group, and the difference was statistically significant (P <0.05). After 3 months of treatment, the levels of N-terminal B-type natriuretic peptide (NT-proBNP) and transforming growth factor β1 (TGF-β1) in two groups were lower than those before treatment, and the levels of 6-minute walking test (6MWT), cardiac output and left ventricular ejection fraction (LVEF) were higher than those before treatment, and which in the observation group were better than those in the control group, the differences were statistically significant (P <0.05). The incidence of adverse reactions in the observation group was lower than that in the control group, and the difference was statistically significant (P <0.05). Conclusion: The clinical effect of Sacubitril Valsartan Sodium in the treatment of elderly patients with ADHF complicated with PAH is more significant than that of Ramipril, which can significantly improve cardiac function, improve serum related indicators, and has good safety. [Key words]　Acute decompensated heart failure　Pulmonary arterial hypertension　Ramipril　Sacubitril Valsartan Sodium First-author's address: Quanzhou First Hospital Affiliated to Fujian Medical University, Quanzhou 362000, China 急性心力衰竭是常见的心血管系统疾病，主要表现为心脏功能迅速出现异常或恶化，同时常伴有血浆利钠肽水平升高。