Definition and Management of Segmental Pulmonary Hypertension.
Definition and Management of Segmental Pulmonary Hypertension.
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DOI：
10.1161/jaha.118.008587
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发表时间：
2018-07-04
影响因子：
5.4
通讯作者：
Gatzoulis MA
中科院分区：
文献类型：
--
作者：
Dimopoulos K;Diller GP;Opotowsky AR;D'Alto M;Gu H;Giannakoulas G;Budts W;Broberg CS;Veldtman G;Swan L;Beghetti M;Gatzoulis MA
(Nice，法国）在儿科和成人患者的分类中引入了一个新的实体，称为“节段性肺动脉高压（PH）”。1节段性PH在2015年国际指南中被描述为“在先天性心脏病（如肺动脉或三尖瓣闭锁）中由肺动脉高压侧支灌注的离散肺区域中观察到的PH”，2而尼斯世界研讨会会议记录1将其定义为“一个或两个肺的一个或多个肺叶中的PH”。其他人将节段性PH更广泛地定义为不遵循均匀分布的PH，肺血管的某些部分暴露于比其他部分更高的压力。3该实体被纳入世界心脏组织第5组（由不清楚或多因素机制引起的PH）的保护伞下，因为对其病理生理学和对肺动脉高压（PAH）治疗的反应知之甚少。1、2节段性PH最常见于先天性心脏病（CHD）患者，与PAH具有显著相似性（第1.4组）。4，PAH伴CHD）和PH分类的第4组（第4.2. 4先天性肺动脉[PA]狭窄患者的PH），但没有系统描述该实体所涵盖的广泛疾病或其独特的病理生理学特征以及这些特征如何影响管理。我们在此提出了一个关于节段性PH的共识声明，包括工作定义、可归类于该实体的条件范围、肺血管病理生理学描述、心血管解剖学和管理原则。
(Nice, France) introduced a new entity in the classification for pediatric and adult patients called “segmental pulmonary hypertension (PH).” 1 Segmental PH was described in the international 2015 guidelines as PH “observed in discrete lung areas perfused by aortopulmonary collaterals in congenital heart diseases such as pulmonary or tricuspid atresia,” 2 while the proceedings of the Nice World Symposium1 defined this as “PH in one or more lobes of one or both lungs.” Others have defined segmental PH more broadly as PH that does not follow a homogeneous distribution, with some parts of the pulmonary vasculature being exposed to higher pressures than others. 3This entity was included under the umbrella of World Heart Organization group 5 (PH caused by unclear or multifactorial mechanisms), because little is known about its pathophysiology and response to pulmonary arterial hypertension (PAH) therapies. 1, 2 Segmental PH is most commonly encountered in patients with congenital heart disease (CHD) and carries notable similarities to PAH (Group 1.4. 4, PAH associated with CHD) and group 4 of the PH classification (Group 4.2. 4 PH in patients with congenital pulmonary artery [PA] stenoses), yet there is no systematic description of the broad spectrum of conditions encompassed by this entity or its distinct pathophysiological features and how these may affect management. We present herewith a consensus statement on segmental PH, including a working definition, range of conditions that may be classified under this entity, description of pathophysiology in terms of pulmonary vasculature, cardiovascular anatomy, and management principles.
居住在高海拔的儿科患者的临床表现和长期随访，具有导管起源的分离肺动脉。
影响因子：
1.6
作者：
Takatsuki, Shinichi;Darst, Jeffrey R.;Das, Bibhuti B.;Fagan, Thomas E.;Wolfe, Robert;Ivy, David Dunbar
通讯作者：
Ivy, David Dunbar