Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children.
Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children.
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DOI：
10.3390/children5040044
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发表时间：
2018-03-23
期刊：
影响因子：
--
通讯作者：
Ivy DD
中科院分区：
文献类型：
--
作者：
Frank BS;Ivy DD
肺动脉高压（PH）是一种肺动脉压力升高的综合征，与受影响儿童的显着发病率和死亡率相关。 PH 与多种潜在的潜在原因相关，包括心脏、肺、血液和风湿病异常。无论原因如何，对于许多患者来说，PH 的自然病程包括肺动脉阻力和压力进行性升高、右心室功能障碍，并最终导致心力衰竭。近年来，许多肺动脉高压（PAH）靶向疗法已可用于降低肺动脉压力并改善预后。成人和儿科文献中越来越多的证据表明，接受治疗的患者的生活质量、功能状态和生存率有所提高。本综述介绍了儿科 PH 的部分病因，并更新了有关 PH/PAH 儿童评估和管理的最新数据。另外还讨论了儿科特定类别 PAH 靶向治疗的现有证据。
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure. In recent years, a number of pulmonary arterial hypertension (PAH)-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome. A growing body of evidence in both the adult and pediatric literature demonstrates enhanced quality of life, functional status, and survival among treated patients. This review provides a description of select etiologies of PH seen in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH/PAH. The available evidence for specific classes of PAH-targeted therapies in pediatrics is additionally discussed.
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影响因子：
168.9
作者：
Berger, Rolf M. F.;Beghetti, Maurice;Humpl, Tilman;Raskob, Gary E.;Ivy, D. Dunbar;Jing, Zhi-Cheng;Bonnet, Damien;Schulze-Neick, Ingram;Barst, Robyn J.
通讯作者：
Barst, Robyn J.
影响因子：
24.3
作者：
Barst, R. J.;Galie, N.;Rubin, L. J.
通讯作者：
Rubin, L. J.
DOI：
10.1111/j.1365-2362.2006.01681.x
发表时间：
2006-09-01
影响因子：
5.5
作者：
Beghetti, M.
通讯作者：
Beghetti, M.
影响因子：
3.4
作者：
Baruteau, Alban-Elouen;Belli, Emre;Bonnet, Damien
通讯作者：
Bonnet, Damien
影响因子：
3.4
作者：
Berger, Rolf M. F.;Gehin, Martine;Bonnet, Damien
通讯作者：
Bonnet, Damien