Pulmonary and Critical Care Division, New England Medical Center, Tufts University School of Medicine, 750 Washington Street, Boston, MA 02111, USA.
Am J Cardiol. 2013 Apr 16;111(8 Suppl):2C-9C. doi: 10.1016/j.amjcard.2013.01.318.
Pulmonary hypertension (PH) is found in many clinical conditions and is associated with increased morbidity and mortality. Pulmonary arterial hypertension (PAH) is a clinical condition characterized by precapillary PH without causes such as lung disease, chronic thromboembolic PH, or other rare conditions. Evaluating a patient with suspected PH requires a series of investigations intended to confirm the diagnosis, determine the clinical PH group (and, in the case of PAH group 1, the specific etiology), and evaluate the functional and hemodynamic impairment. The workup should identify the risk factors for PH (e.g., left heart disease, lung diseases associated with alveolar hypoxia, and chronic thromboembolism) versus the conditions associated with PAH group 1 (e.g., scleroderma, human immunodeficiency virus, anorexigen use, liver disease). A detailed algorithm is presented to help physicians determine the appropriate PH category. Because the presence of one condition associated with PH does not exclude another etiologies, clinicians are strongly encouraged to follow the entire algorithm. Discussions and case studies are presented describing the differentiation of PAH group 1 from PH group 2 and PAH group 1 from PH group 3; diagnosing PH group 4; determining the long-term calcium channel blocker response in those with idiopathic PAH; and determining the severity of PH.
肺动脉高压（PH）存在于许多临床情况下，与发病率和死亡率的增加相关。肺动脉高压（PAH）是一种以毛细血管前 PH 为特征的临床病症，没有肺疾病、慢性血栓栓塞性 PH 或其他罕见病症等原因。评估疑似 PH 的患者需要一系列检查，旨在确认诊断、确定临床 PH 组（对于 PAH 组 1，确定特定病因），并评估功能和血流动力学损伤。该检查应确定 PH 的风险因素（例如，左心疾病、与肺泡缺氧相关的肺部疾病和慢性血栓栓塞）与 PAH 组 1 相关的病症（例如，硬皮病、人类免疫缺陷病毒、厌食症药物使用、肝病）。提出了详细的算法来帮助医生确定适当的 PH 类别。由于存在一种与 PH 相关的病症并不排除其他病因，因此强烈鼓励临床医生遵循整个算法。讨论和案例研究介绍了如何区分 PAH 组 1 与 PH 组 2 和 PAH 组 1 与 PH 组 3；诊断 PH 组 4；确定特发性 PAH 患者中长效钙通道阻滞剂的反应；以及确定 PH 的严重程度。
