Pulmonary hypertension: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.

Pulmonary hypertension: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.
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DOI:
10.1513/annalsats.201312-443ld
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发表时间:
2014-04-01
影响因子:
8.3
通讯作者:
Abman, Steven H
Abman, Steven H
中科院分区:
医学1区
文献类型:
--
作者:
Austin, Eric D;Kawut, Steven M;Abman, Steven H

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肺血管功能障碍(PVD)先于肺动脉高压(PAH)的临床体征和症状发作。PAH定义为肺动脉压升高,通常进展为右心室(RV)功能障碍和衰竭。PAH影响所有年龄段的受试者,并与多种医学疾病相关,其中大多数是罕见的。有几个因素对PAH一级预防策略的制定构成了直接挑战,包括:(1)特发性或原发性疾病极为罕见,限制了筛查的实用性;(2)目前尚未建立临床前PVD的检测方法;(3)对正常和PAH状态下肺血管生长、结构和功能的决定因素的理解不足;(4)可用于长期研究以准确评估疾病发展的“有风险”受试者的数量相对较少;以及(5)缺乏PVD的预防性疗法。尽管存在这些局限性,但利用已知的高危患者人群进行研究,以及从系统生物学到先进和更敏感的功能成像模式等多个学科的不断发展,可能有助于在未来十年内显着改善一级预防研究和实施。
Pulmonary vascular dysfunction (PVD) precedes the onset of clinical signs and symptoms of pulmonary arterial hypertension (PAH). PAH is defined by the elevation of pulmonary arterial pressure, which often progresses to right ventricular (RV) dysfunction and failure. PAH affects subjects of all ages, and is associated with diverse medical conditions, most of which are rare. Several factors pose immediate challenges to the development of strategies for primary prevention of PAH, including: (1) the idiopathic or primary form of the disease is extremely rare, limiting screening practicality; (2) methods for the detection of preclinical PVD are currently not established; (3) the understanding of determinants of pulmonary vascular growth, structure, and function in normal and PAH states is insufficient; (4) relatively small numbers of "at-risk" subjects are available for long-term studies to accurately assess disease development; and (5) preventative therapies for PVD are lacking. Despite these limitations, leveraging known at-risk patient populations for study, as well as growing progress across multiple disciplines, ranging from systems biology to advanced and more sensitive functional imaging modalities, may facilitate the opportunity to significantly improve primary prevention research and implementation over the next decade.