Severe pulmonary hypertension in lung disease: phenotypes and response to treatment
Severe pulmonary hypertension in lung disease: phenotypes and response to treatment
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DOI：
10.1183/13993003.02307-2014
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发表时间：
2015-11-01
影响因子：
24.3
通讯作者：
Peacock, Andrew J.
中科院分区：
文献类型：
--
作者：
Brewis, Melanie J.;Church, Alistair C.;Peacock, Andrew J.
肺部疾病引起的肺动脉高压（PH）是常见的（世界卫生组织（WHO）第3组），但严重的PH（任意定义为平均肺动脉压>= 35 mmHg）只有一小部分报道。这些患者是否应作为WHO第1组（即肺动脉高压）患者接受ph靶向治疗尚不清楚。我们比较了118例严重PH和肺部疾病患者与74例特发性肺动脉高压（IPAH）患者的表型特征和结果，所有患者均接受肺血管扩张剂治疗。肺部疾病患者比IPAH患者年龄更大，低氧血症更严重，气体传递更低，纽约心脏协会功能等级更差，6分钟步行距离（6MWD）更短。肺疾病患者较差的生存率是由间质性肺疾病（ILD）队列驱动的。与IPAH相比，在IPAH中，6MWD和n -末端前脑自然肽（NT-proBNP）发生了显著改善，PH治疗严重PH肺病并没有导致6MWD或功能等级的改善，但也没有出现恶化。NT-proBNP从2200降至1596 pg.mL(-1) （p=0.015）。反应因肺部疾病表型而异，ILD和肺气肿患者的预后较差，并保留1 s的用力呼气量。需要进一步研究血管扩张剂治疗是否可以延缓严重PH伴肺部疾病的疾病进展。
Pulmonary hypertension (PH) due to lung disease (World Health Organization (WHO) group 3) is common, but severe PH, arbitrarily defined as mean pulmonary artery pressure >= 35 mmHg is reported in only a small proportion. Whether these should be treated as patients in WHO group 1 (i.e. pulmonary arterial hypertension) with PH-targeted therapies is unknown.We compared the phenotypic characteristics and outcomes of 118 incident patients with severe PH and lung disease with 74 idiopathic pulmonary arterial hypertension (IPAH) patients, all treated with pulmonary vasodilators.Lung disease patients were older, more hypoxaemic, and had lower gas transfer, worse New York Heart Association functional class and lower 6-min walking distance (6MWD) than IPAH patients. Poorer survival in those with lung disease was driven by the interstitial lung disease (ILD) cohort.In contrast to IPAH, where significant improvements in 6MWD and N-terminal pro-brain natruiretic peptide (NT-proBNP) occurred, PH therapy in severe PH lung disease did not lead to improvement in 6MWD or functional class, but neither was deterioration seen. NT-proBNP decreased from 2200 to 1596 pg.mL(-1) (p=0.015). Response varied by lung disease phenotype, with poorer outcomes in patients with ILD and emphysema with preserved forced expiratory volume in 1 s. Further study is required to investigate whether vasodilator therapy may delay disease progression in severe PH with lung disease.