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肺动脉高压:不明原因呼吸困难的罕见病因

[Pulmonary hypertension: a rare cause of unexplained dyspnea].

作者信息

Vachiéry J L

机构信息

Clinique de l'Hypertension pulmonaire et de l'Insuffisance cardiaque, Service de Cardiologie, Hôpital Erasme, Bruxelles.

出版信息

Rev Med Brux. 2012 Sep;33(4):280-2.

Abstract

Pulmonary hypertension (PH) is defined by an increase in mean pulmonary artery pressure above 25 mmHg, measured at right heart catheterization. The various conditions (up to 37) leading to PH are described in a clinical classification identifying 5 groups, including pulmonary arterial hypertension (PAH). With an incidence of 2-4 cases/million/year, PAH is a rare, rapidly progressive and incurable form or PH. The differential diagnosis of PH relies on a decision tree, which is typically triggered by the presence of unexplained dyspnea and followed by a non invasive approach that includes simple tests such as EKG, chest radiography, pulmonary function tests and echocardiography. Other tests have some value to exclude chronic thromboembolic pulmonary hypertension, such as ventilation/perfusion scintigraphy, angio CT scanner and pulmonary angiogram. Finally, right heart catheterization is mandatory to establish the diagnosis of PH.

摘要

肺动脉高压(PH)定义为通过右心导管检查测得的平均肺动脉压升高至25 mmHg以上。导致PH的各种情况(多达37种)在一项临床分类中进行了描述,该分类确定了5组,包括肺动脉高压(PAH)。PAH的发病率为每年2 - 4例/百万人,是一种罕见、进展迅速且无法治愈的PH形式。PH的鉴别诊断依赖于一个决策树,通常由不明原因的呼吸困难引发,随后采用非侵入性方法,包括如心电图、胸部X线摄影、肺功能测试和超声心动图等简单检查。其他检查对于排除慢性血栓栓塞性肺动脉高压有一定价值,如通气/灌注闪烁扫描、血管CT扫描仪和肺血管造影。最后,必须进行右心导管检查以确诊PH。

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